Download SELECTED CASES

2016
Bridging the World of Endocrinology
20 - 23 October 2016
Cornelia Diamond Hotel, Antalya - Turkey
SELECTED CASES
w w w . e n d o b r i d g e . o r g
2016
SCIENTIFIC PROGRAM
Friday, 21 October 2016
08.40-09.00 Welcome and Introduction to EndoBridge 2016
MAIN HALL
09.00-09.30
09.30-10.00
10.00-10.30
10.30-11.00
Chairs: Jens Bollerslev, Refik Tanakol
Recent advances in clinical and molecular understanding of metabolic bone disease - Nilgün Güvener Demirağ
Approach to “drug holidays” “treatment failures” and possible combination therapy for postmenopausal
osteoporosis Hasan Aydın
Medical therapy in primary hyperparathyroidism - Peter Schwarz
Update on Vitamin D - Skeletal and nonskeletal effects - Ghada El-Hajj Fuleihan
11.00-11.20
COFFEE BREAK
11.20-12.50 HALL A
HALL B
HALL C
HALL D
Interactive Case Discussion Sessions
Bone / Calcium - Jens Bollerslev, Alper Gürlek
Thyroid - Ralf Paschke, Seda Sancak
Obesity / Lipids - Marc Cornier, Oğuzhan Deyneli
Male Reproductive Endocrinology - Joshua Safer, Andrea Isidori
LUNCH
12.50-14.00
14.00-15.30 HALL A
HALL B
HALL C
HALL D
Interactive Case Discussion Sessions
Bone / Calcium - Peter Schwarz, Ghada El-Hajj Fuleihan
Thyroid - Michael McDermott , Georg Brabant
Obesity / Lipids - Marc Cornier, Sinem Küçüksaraç Kıyıcı
Female Reproductive Endocrinology - Duarte Pignatelli, Füsun Saygılı
see page 5 for details
COFFEE BREAK
15.30-15.50
MAIN HALL
15.50-16.20
16.20-16.50
16.50-17.20
17.20-17.50
see page 5 for details
Chairs: Georg Brabant, M. Sait Gönen
Perplexing thyroid function tests - Michael McDermott
Management of subclinical thyroid dysfunction including pregnancy - Georg Brabant
Molecular diagnostic testing in evaluation of thyroid nodules - Ralf Paschke
Differentiated thyroid cancer: Which type of surgery? How much RAI? How much suppression? - Ayşe Kubat Üzüm
20 - 23 October, 2016
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Antalya - Turkey
2016
SCIENTIFIC PROGRAM
Saturday, 22 October 2016
MAIN HALL
09.00-09.30
09.30-10.00
10.00-10.30
10.30-11.00
Chairs: Camilla Schalin-Jäntti, Bülent Yıldız
Management of hyperglycemia and cardiovascular disease in type 2 diabetes - Rita Kalyani
What is new in insulin therapy of diabetes? - Richard Holt
Diabetes technologies: Current use and future perspectives Hans De Vries
Bariatric-metabolic surgery in type 2 diabetes - Kåre Birkeland
COFFEE BREAK
11.00-11.20 11.20-12.50
HALL A
HALL B
HALL C
HALL D
Interactive Case Discussion Sessions
Diabetes - Kåre Birkeland, Rita Kalyani
Adrenal - Massimo Terzolo, Özlem Çelik
Pituitary - AJ Van Der Lely, Nurperi Gazioğlu
Neuroendocrine Tumors - Camilla Schalin-Jäntti, Eva Tiensuu Janson
LUNCH
12.50-14.00
14.00-15.30 HALL A
HALL B
HALL C
HALL D
Interactive Case Discussion Sessions
Diabetes - Hans De Vries, Mohamed Hassanein
Adrenal - Jerome Bertherat, Tevfik Demir
Pituitary - Vera Popovic, John Wass
Neuroendocrine Tumors - Camilla Schalin-Jäntti, Eva Tiensuu Janson
COFFEE BREAK
15.30-15.50 MAIN HALL
15.50-16.20
16.20-16.50
16.50-17.20
17.20-17.50
see page 6 for details
Chairs: George Mastorakos, Fahri Bayram
The “omics” of adrenocortical cancer - Jerome Bertherat
Approach to adrenal incidentaloma: 2016 guidelines -Massimo Terzolo
Male hypogonadism - Andrea Isidori
Care of transgender patients - Joshua Safer
20 - 23 October, 2016
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Antalya - Turkey
see page6 for details
2016
SCIENTIFIC PROGRAM
Sunday, 23 October 2016
MAIN HALL
09.00-09.30
09.30-10.00
10.00-10.30
10.30-11.00
Chairs: AJ Van der Lely, Melek Eda Ertörer
Evaluation and management of sellar masses - Vera Popovic
Can we really cure Cushing’s disease? - Susan Webb
Medically refractory prolactinoma - John Wass
Challenges and controversies in diagnosis and management of acromegaly - Pınar Kadıoğlu
COFFEE BREAK
11.00-11.20
MAIN HALL
11.20-11.50
Chairs: Marja-Riitta Taskinen, İlhan Yetkin
Abdominal obesity, type 2 diabetes and cardiometabolic health - Amika Singh
* Supported by International Chair on Cardiometabolic Risk
11.50-12.20
Ectopic fat: A new target for CVD risk management - Marja-Riitta Taskinen
* Supported by International Chair on Cardiometabolic Risk
12.20-12.50
12.50-13.20
Food addiction: Does it really exist? - AJ Van der Lely
The evolving science of nutrition and nutritional quality- Reyhan Nergiz Ünal
13.20-13.30 Closing comments and adjourn
20 - 23 October, 2016
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2016
SELECTED CASES
DATE
TIME
HALL
SESSION
TITLE
21.10
11.20-12.50
A
Bone/Calcium
O-01 / Postoperative Hypoparathyroidism: Treatment With
Teriparatide
21.10
11.20-12.50
A
Bone/Calcium
O-02 / A case of primary hyperparathyroidism diagnosed
during pregnancy
O-03 / A striking finding during intraoperative jugular
sampling in a case of parathyroid adenoma with low-normal
serum intact parathyroid hormone levels
21.10
11.20-12.50
A
Bone/Calcium
21.10
11.20-12.50
B
Thyroid
O-04 / A case of hypothyroidism requiring parenteral
levothyroxine treatment
21.10
11.20-12.50
B
Thyroid
O-05 / Primary thyroid lymphoma presenting with subacute
thyroiditis
21.10
11.20-12.50
B
Thyroid
O-06 / A rare case of thyrotoxic periodic paralysis with
accompanying transient hyperglycemia
21.10
11.20-12.50
B
Thyroid
O-07 / A case with MEN-2b who has neurinoma of the
tongue and eyes
21.10
11.20-12.50
B
Thyroid
O-08 / A case of papillary thyroid carcinoma in toxic
adenoma: Are hyperfunctioning nodules truly innocent all
the times?
21.10
11.20-12.50
B
Thyroid
O-09 / Medically refractory amiodarone-induced
thyrotoxicosis: Two case reports
21.10
11.20-12.50
C
Lipids/Obesity
O-10 / Metabolic abnormalities associated with insulin
resistance in a young patient with acquired partial
lipodystrophy
21.10
14.00-15.30
A
Bone/Calcium
O-12 / Effect of zolendranate treatment on hearing loss in a
patient with the Paget’s disease who has skull involvement
21.10
14.00-15.30
A
Bone/Calcium
O-13 / Thyroid nodule or parathyroid adenoma: a case report
21.10
14.00-15.30
B
Thyroid
O-14 / A case of thrombotic thrombocytopenic purpura in a
toxic multinodular goiter patient on antithyroid medication
21.10
14.00-15.30
B
Thyroid
O-15 / Agressive follicular variant of papillary
microcarcinoma presenting with extensive bone metastases
and associated with weird thyroid function tests
21.10
14.00-15.30
B
Thyroid
O-16 / Thyrotoxic Periodic Paralysis
21.10
14.00-15.30
B
Thyroid
O-17 / Thyrotoxicosis with pleural effusion and
hypoalbuminemia
21.10
14.00-15.30
B
Thyroid
O-18 / Plasmapheresis as a temporary management option
in a patient with Graves’ disease and primary sclerosing
cholangitis
21.10
14.00-15.30
B
Thyroid
O-19 / A lump on the scalp: An unusual presentation of
papillary thyroid carcinoma
21.10
14.00-15.30
C
Lipids/Obesity
O-20 / Wernicke encephalopathy in a case who had gastric bypass
surgery due to morbid obesity
20 - 23 October, 2016
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2016
DATE
TIME
HALL
SESSION
TITLE
22.10
11.20-12.50
A
Diabetes
O-21 / The investigation for celiac disease in a newly
diagnosed patient with diabetes is necessary in any case
22.10
11.20-12.50
A
Diabetes
O-22 / Mauriac syndrome still exists; a rare complication of
type 1 diabetes mellitus
22.10
11.20-12.50
B
Adrenal
O-23 / Unilateral adrenalectomy improves cushing
syndrome in a patient with primary bilateral macronodular
adrenal hyperplasia
22.10
11.20-12.50
B
Adrenal
O-24 / CAH presenting as premature puberty with associated
testicular adrenal rest tumors (TART)
22.10
11.20-12.50
B
Adrenal
O-25 / Different clinical presentations in three patients with
adrenocortical cancer
22.10
11.20-12.50
B
Adrenal
O-26 / Bilateral cystic hemorrhagic adrenal incidentalomas
22.10
11.20-12.50
B
Adrenal
O-27 / Adrenocortical carcinoma presenting with Cushing’s
syndrome in a patient with 8 years history of adrenal
insufficiency and adrenomyolipomas
22.10
11.20-12.50
C
Pituitary
O-28 / Idiopathic granulomatous hypophysitis mimicking
pituitary adenoma
22.10
11.20-12.50
C
Pituitary
O-29 / Case of giant prolactinoma in young man
22.10
11.20-12.50
C
Pituitary
O-30 / Pituitary stalk interruption syndrome: a rare cause of
panhypopituitarism
22.10
11.20-12.50
D
Neuroendocrine
tumors
O-31 / How multifocal insulinomas can be diagnosed and
treated in a case with MEN-1 disease?
22.10
11.20-12.50
D
Neuroendocrine
tumors
O-32 / Catastrophic Cushing’s Syndrome: Report of a mortal
case
22.10
14.00-15.30
A
Diabetes
O-33 / Effect of Imatinib on insulin therapy in a patient with
Type 2 DM
22.10
14.00-15.30
B
Adrenal
O-35 / A Case Of Von Hippel-Lindau Disease with bilateral
pheochromocytoma, abdominal paraganglioma and
pancreatic neuroendocrine tumor
22.10
14.00-15.30
B
Adrenal
O-36 / Silent Pheochromocytoma in von Hippel-Lindau
Disease: Report of a Case
22.10
14.00-15.30
B
Adrenal
O-37 / Adrenocortical carcinoma in pregnancy
22.10
14.00-15.30
B
Adrenal
O-38 / A case with adrenal cyst hydatid mimicking malignant
tumour in MRI and PET-CT
22.10
14.00-15.30
C
Pituitary
O-39 / Hamartoma of hypothalmus presented as precocious
puberty and epilepsy in 10-years old girl
22.10
14.00-15.30
C
Pituitary
O-40 / A rare cause of Diabetes Insipidus: Rosai Dorfman
Disease
22.10
14.00-15.30
D
Neuroendocrine
tumors
O-41 / Localization of an insulinoma by selective
intraarterial calcium stimulation with hepatic venous
sampling
22.10
14.00-15.30
D
Neuroendocrine
tumors
O-42 / Known syndrome, a new case
20 - 23 October, 2016
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2016
ORAL
PRESENTATIONS
20 - 23 October, 2016
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2016
O - 01
Bone/Calcium
Postoperative Hypoparathyroidism:
Treatment With Teriparatide
Deniz Can Güven1, Ömer Alper Gürlek2, Seda Oğuz2, Nafiye Helvacı2
1Deparment of Internal Medicine, Hacettepe University Medical Faculty, Ankara, Turkey
2Deparment of Endocrinology, Hacettepe University Medical Faculty, Ankara, Turkey
Postoperative hypoparathyroidism is a common problem, following both thyroid and parathyroid surgeries. Conventionally vitamin D and elementary calcium have been the mainstay of therapy.
Herein, we present our experience with teriparatide (recombinant
parathormone) in a postoperative hypoparathyroidism patient.
A 28-year-old female patient was referred to our clinic due to euthyroid autoimmune thyroiditis with thyroid nodules. The patient was
followed without treatment. Three years later, the patient applied to
our clinic with inflammatory low back pain and morning stiffness.
An MRI of sacroiliac joint showed bilateral grade III sacroileitis. After the patient’s evaluation with these findings, the diagnosis was
made as ankylosing spondylitis. Before starting the steroid treatment, a bone mineral density measurement was performed and
showed a lumbar spine Z score of -6.6 and femur Z score of -4. Total calcium and phosphate levels were 10.5 mg/dL and 2.5 mg/dL,
respectively. Serum PTH test was ordered and it was 1619 pg/ml.
Parathyroid ultrasonography revealed two lesions located inferior
of each thyroid lobe which were compatible with parathyroid gland
pathology. Total thyroidectomy and parathyroid gland excision and
servical lymph node dissection were performed with the preliminary diagnosis of parathyroid carcinoma. Histopathological examination of surgical specimen showed chronic lymphocytic thyroiditis
and parathyroid adenoma. In the postoperative period, the patient’s
total calcium levels was around 5.5-6 mg/dL. IV calcium replace-
20 - 23 October, 2016
ment with oral calcitriol and calcium carbonate was required for
three weeks postoperatively. After discharge with a maintenance
regimen of calcitriol 12 mcg/day, calcium carbonate 15 grams/day
and cholecalciferol 3600 IU/day, the patient’s hypocalcemia persisted and hyperphosphatemia developed. At this point we decided to
start subcutaneous recombinant parathyroid hormone. Teriparatide (20 μg/12 h s.c.) was started. This therapy enabled dose reduction of calcitriol down to 4.5 mcg/day and calcium carbonate to 9
grams/day. Hyperphosphatemia was resolved. After seven months
of therapy teriparatide dose was tapered to a daily single dose
which was ceased completely after another month. Hypocalcemia
did not recur after the cessation of teriparatide. During 5 years
of follow up, the patient has been normocalcemic under the regimen of calcitriol 1.5 mcg/day and calcium carbonate 3 grams/day.
Short term use of teriparatide is reported in multiple case reports
especially in patients with thyroidectomy and neck dissection. A
prospective study with four years follow up provided data for the
safety and efficacy of long term teriparatide treatment in postoperative hypoparathyroidism. Although further studies are required
for long term effects, teriparatide seems to be a good choice with
a favorable safety profile for refractory postoperative hypoparathyroidism.
Keywords: refractory postoperative hypoparathyroidism, teriparatide, parathyroid adenoma
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2016
O - 02
Bone/Calcium
A case of primary hyperparathyroidism
diagnosed during pregnancy
Hakan Doğruel, Nusret Yılmaz, Özlem Doğan, Hümeyra Bozoğlan, Ramazan Sarı,
Hasan Ali Altunbaş, Mustafa Kemal Balcı
Akdeniz University Hospital, antalya, turkey
Primary hyperparathyroidism (PHPT) is a rare disorder. Incidence
is 8/100000.This incidence is same among child bearing age women and pregnants. PHPT during pregnancy is associated with several risks to the mother and fetus especially when maternal total
serum calcium is >11 mg/dL. In gestational PHPT cause is a single
adenoma in %80-85 patients, hyperplasia in %10-12 patients, multiple adenoma in %2 patients and carcinoma %1 patients as general population. We present a case of PHPT diagnosed during third
trimester of pregnancy.
required several times because of hypercalcemia during pregnancy
and normocalcemia was enabled with intraveneous saline infusion.
She gave birth to a hypotonic and hypoglycemic male infant at 37th
week of gestatonal age with caesarean section. Baby’s serum calcium level was 10,5 mg /dl and PTH level was 1 pg/ml after birth.
Mother’s serum calcium level was 11.6 before labor and normocalcemic state was also enabled with intraveneous saline infusion.
Her serum calcium level is 10,2 mg/dl at postpartum follow up. We
planned surgical operation later on.
A 43 years old woman, was under follow up of obstetric clinic because of her fourth pregnancy. She was admitted to the hospital
with abdominal pain, nausea and constipation at 27th week of gestational age. Complete blood count and biochemical tests were totally normal except hypercalcemia [Ca:11,4 mg/dl (8,7-10,4)]. She
was referred to our clinic because of hypercalcemia. There were
no pathological findings on her physical examination. Hyperparathyroidism was detected with initial tests for differential diagnosis; creatinine:0,62 mg/dl (0,5-11), Ca: 11,4 mg /dl (8,7-10,4),P:
2,67 mg/dl(2,4-5,5), PTH: 140 pg/ml (14-72),25-OH D3:18,2 ng/ml
(30-100), 24 hour urine Ca:310 mg/24h (<300). An adenoma like
hypoechoic lesion (image1) with size of 19 X 6 X 15 mm was detected with ultrasound on right inferior thyroid region. We didn’t
plan surgery because she was in the third trimester of pregnancy.
We planned close follow up during pregnancy. Hospitalization was
Keywords: pregnancy, hyperparathyroidism, pregnancy and hyperparathyroidism, hypercalcemia and pregnancy
20 - 23 October, 2016
Figure 1. ultrasound view of parathyroid adenoma
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O - 03
Bone/Calcium
A striking finding during intraoperative
jugular sampling in a case of parathyroid
adenoma with low-normal serum intact
parathyroid hormone levels
Ilgın Yıldırım Şimşir1, Banu Şarer Yürekli1, Hatice Özışık1, Murat Özdemir2,
Yeşim Ertan3, Özer Malay2, Gökhan Özgen1
1 Ege University Faculty of Medicine, Department of Endocrinology, Izmir, Turkey
2 Ege University Faculty of Medicine, Department of General Surgery, Izmir, Turkey
3 Ege University Faculty of Medicine, Department of Pathology, Izmir, Turkey
Aim: Primary hyperparathyroidism (PHP) is the most common
cause of hypercalcemia based on outpatient clinic. The diagnosis
of PHP is generally straightforward with high intact PTH (iPTH) and
hypercalcemia. But, PHP does not always exhibit those biochemical features. Less known phenotype of PHP is the hypercalcemia
with normal level of iPTH. We report a case of parathyroid adenoma
with low normal peripheral iPTH and high jugular PTH levels presenting as hypercalcemia.
Case: A thirty-six year old woman who have type 2 diabetes mellitus was admitted to the hospital with the diagnosis of acute pancreatitis. She had no cholelithiasis. Serum amylase and lipase levels
were high. Acute edematous pancreatitis was shown on computed tomography. She had hypertriglyceridemia in her past medical
history. Serum of the patient was observed as lipemic. Hypertriglyceridemia could be a reason for the pancreatitis. On laboratory
examination at admission to the hospital; calcium level was 8.0 mg/
dL (8.8-10.2), intact PTH (iPTH) level was 28.8 pg/mL (12-88) and
25-hydroxy vitamin D level was 6.5 ng/mL. Cholecalciferol was given intramuscularly as 300.000 IU. In her past medical history, hypothyrodism was present. Thyroid ultrasonography was performed.
There was a hypoechoic solid nodule on right thyroid lobe sized as
7x3mm. There was also 17x6 mm sized lesion at the lower part of
right thyroid lobe that revealed as possible parathyroid adenoma.
On Tc 99m MIBI scintigraphy, a lesion was seen at the lower part of
20 - 23 October, 2016
right thyroid lobe (Figure 1). This lesion was thought as parathyroid
adenoma. There was no osteoporosis according to bone mineral
densitometry. After replacement of vitamin D and acute pancreatitis subsided, serum Ca and iPTH levels were as follows: 10.6, 10.7,
10.9 mg/dL and 28.8, 33.3, 31.7 pg/mL, respectively. Twenty-four
hour calcium excretion was 403 mg/day, serum phosphorus was
2.3 mg/dL. iPTH measurement was repeated in another accredited
laboratory and was found as 27 pg/mL. iPTH was measured also by
using dilution technique and obtained as 38 pg/mL. Hydration and
furosemide treatment were performed, but Ca level was still high,
which was 11.4 mg/dL. Besides hypertriglyceridemia, we thought
that hypercalcemia could precipitate the episodes of pancreatitis.
Based on the findings of hypercalcemia, hypercalciuria, positive sonographic and scintigraphic images, operation was performed with
the possible diagnosis of PHP. PTH measurement was taken from
the right jugular vein. Intraoperative PTH was 634 pg/mL. Histopathological examination revealed as parathyroid adenoma. Postoperative hypocalcemia didn’t occur and postoperative calcium and
iPTH levels were normal.
Conclusion: Clinicians should be aware of the presence of low-normal iPTH in PHP. If the clinical suspicion of PHP is high as in our
case, surgery should be performed after exclusion of other causes
of hypercalcemia.
Keywords: Hypercalcemia, low-normal PTH, parathyroid adenoma
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2016
O - 04
Thyroid
A case of hypothyroidism requiring
parenteral levothyroxine treatment
Hande Peynirci1, Bengür Taşkıran2, Erdinç Ertürk3, Pınar Şişman3, Canan Ersoy3
1 Kastamonu State Hospital, Kastamonu, Turkey
2 Yunus Emre State Hospital , eskişehir, Turkey
3 Uludag University , bursa, Turkey
Introduction: Some hypothyroid patients need high doses of oral
levothyroxine (LT4) due to poor adherence to treatment, not taking the
drug on an empty stomach, malabsorption, and co-administration of
drugs interfering with absorption. We present a case of isolated malabsorption of LT4 responding to intramuscular (IM) therapy.
Clinical Case: A 32 year old female patient was admitted to hospital
due to hair loss and massive weight gain in 2008. She had thyroidectomy and radioactive iodine therapy in 2004. She was on 400 μg/d LT4
therapy. Physical examination revealed dry skin, non-pitting oedema,
and diminished deep tendon reflexes. History of systemic diseases,
diarrhea, and co-administration of any other drugs or herbal products
interfering with LT4 absorption were absent. Thyroid function tests
(TFT) did not improve despite medication under supervision.
Gastrointestinal endoscopy and stool analysis was normal. Antiparietal, endomysium, and transglutaminase antibodies were negative.
Triiodothyronine added to high-dose LT4 therapy was ineffective.
To evaluate LT4 malabsorption, 1000 µg LT4 was administered
orally and TFT were measured with 2 hour intervals for 6 hours.
FT4 level did not change. During the test she developed aphasia,
loss of vision, and hemiparesis. Cranial imaging was normal and
transient ischemic attack (TIA) was diagnosed. The neurologic
symptoms regressed with anti-oedema and acetylsalicylic acid
(ASA) therapy. She was discharged with daily 450 µg LT4 and 75 µg
triiodothyronine therapy.
After euthyroidism was achieved, parenteral route was switched to
oral route according to the assumption that malabsorption due to
oedema in the gastrointestinal tract secondary to hypothyroidism
might have improved. However hypothyroidism ensued soon. Previous regime was started again. She was euthyroid at the last time
she was seen.
Conclusion: Impaired LT4 absorption may continue despite vigorous
acts against factors interfering with absorption. Due to its limited marketing and its high cost, LT4 is administered for short term via slow
IV infusion. The only risk for TIA and atherosclerosis was prolonged
hypothyroidism. She did not experience acute variations in serum T4
during high dose LT4 therapy to explain to TIAs. An adverse effect of
high dose LT4 therapy seems to be the best explanation for TIAs.
Keywords: hypothyroidism, malabsorption, parenteral levothyroxine, transient ischemic attack
Table 1. The changes in the levels of free throxine and thyroid stimulating hormone after administration of intramuscular 500 µg LT4 twice
a week (FT4: Free thyroxine, TSH:Thyroid stimulating hormone)
She was lost to follow-up until 2012. During this period she had
another TIA during intravenous (IV) therapy in another center. Furthermore rectal route could not be tolerated and vaginal route was
unsuccessful.
Her treatment schedule was planned as weekly increased doses of
200, 500, 1000, and 1200 µg IM LT4. TFT were measured at weekly intervals. She developed left sided loss of strength during 1200 µg dose.
Cranial imaging was again normal. The symptoms regressed within a
week with ASA therapy. Detailed tests for coagulopathy were normal.
TFT quickly normalized with IM 500 µg LT4 twice a week. No other
TIAs occurred.
20 - 23 October, 2016
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2016
Table 2. The changes in the levels of free T4 and TSH after administration of oral 1000 mcg LT4 at the end of a 6-hour monitoring
TFT
baseline
2nd hour
4th hour
6 th hour
FT4 (ng/dL)
0.40
0.40
0.40
0.40
TSH (μIU/mL)
100
81.81
84.74
93.48
20 - 23 October, 2016
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2016
O - 05
Thyroid
Primary thyroid lymphoma presenting with
subacute thyroiditis
Sevgül FakI1, Neslihan Çuhacı1, Oya Topaloğlu1, Cüneyt Bilginer1, Berna Öğmen1,
Hakan Korkmaz2, Ünsal Han3, Reyhan Ersoy1, Bekir Çakır1
1 Department of Endocrinology and Metabolism, Ankara Yildirim Beyazit University, School of Medicine, Ankara,
Turkey
2 Department of Otorhinolaryngology, Ankara Yildirim Beyazit University, School of Medicine, Ankara, Turkey
3 Department of Pathology, Ankara Yildirim Beyazit Education and Research Hospital, Ankara, Turkey
Background: Primary thyroid lymphoma (PTL) is defined as a lymphoma involving only the thyroid gland or the thyroid gland and adjacent
(regional) neck lymph nodes. Thyroid lymphoma represents 4% of all
malignancies that is a relatively rare disease often posing a diagnostic
challenge (1). Reaching the final diagnosis can be delayed if insufficient
biopsy material is obtained. FNA diagnosis of thyroid lymphoma, particularly due to the histological similarities with thyroiditis and the high
coincidence of these pathologies within the same gland, which results
in increased false-negative rates from sampling error (2). The present
study describes the case of a patient who was treated as subacute thyroiditis and diagnosed as having diffuse large B-cell lymphoma.
Clinical Case: A 61-year-old female presented with anterior neck
pain, hoarseness and rapidly expanding mass of the neck diagnosed
as subacute thyroiditis and she had recieved NSAİ at least 2 months
for her pain 4 months earlier. Fine needle aspiration cytodiagnosis
was benign. The patient was admitted to our hospital because of increasing dyspnea. Laboratory studies revealed an euthyroid state;
antithyroglobulin and thyroid peroxidase antibodies were both positive. In PA chest X-ray trachea was narrowed and shifted to the left
side. A neck ultrasound showed a significantly enlarged, diffuse parenchymal inhomogeneity and ill defined border over right lobe and
isthmus of the thyroid gland. Also, the trachea was narrowed and
pressed to the right. Tru-cut biopsy was performed and histological
diagnosis was Diffuse large B-cell lymphoma of the thyroid gland.
A computed tomography scan demonstrated that the trachea was
markedly compressed and displaced by the mass. His respiratory
status was so urgent that the patient was given corticosteroids and
saved her from receiving a tracheostomy. After she recieved chemotherapy she was in a good physical condition.
Conclusion: The most common presentation of subacute thyroiditis is an anterior neck pain radiating up to the jaw and ear. Other
causes of pain in the thyroid gland should be taken into consideration during differential diagnosis, especially when a patient presents with misleading symptoms like progressive dyspnea, dysphagia or dysphonia. Such a presentation should be acknowledged and
this leads to early diagnosis, treatment and prevention of unnecessary surgery.
20 - 23 October, 2016
Keywords: Primary thyroid lymphoma, subacute thyroiditis, neck
mass
Figure 1: A computed tomography
Figure 1. A computed tomography
the trachea was markedly compressed and displaced by the mass.
Figure 2: PA chest X-ray
Figure 2. PA chest X-ray yazılacak
narrowing and shifting of trachea
13
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2016
0 - 06
Thyroid
A rare case of thyrotoxic periodic paralysis
with accompanying transient hyperglycemia
BaŞak Özgen Saydam, Ümit Çavdar, Barış Akıncı, Tevfik Demir
Department of Endocrinology and Metabolism, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey
Introduction:Thyrotoxic periodic paralysis (TPP) is an uncommon
complication of thyrotoxicosis which is associated with paralysis
and hypokalemia. Treatment of TPP consists of adequate potassium replacement, beta-adrenergic blockage and control of hyperthyroidism.
Clinical Case: A-51-year-old male patient was admitted to emergency department with complaint of muscle weakness which started 2 weeks ago but was aggravated a high carbohydrate intake.
He was admitted to department of cardiology in another hospital
3 months ago because of dyspnea and his coronary angiography
showed normal coronaries. He had no history of diabetes or hypertension. One week after coronary angiography he was applied to
endocrinology and metabolism unit with complaint of sweating and
subclinical hyperthyroidism was diagnosed. Non-iodized salt was
recommended. In his admission to emergency department in our
hospital; his physical examination revealed proximal muscle weakness in both upper extremities (4-/5) and lower extremities (2/5).
His electrocardiogram showed u waves with 90 beats per minute.
Other physical examination findings were unremarkable. His biochemical analysis results were as follows: random plasma glucose:
363mg/dl, serum creatinine: 0.53mg/dL, sodium: 138mmol/L,
potassium: 1.9mmol/L, total calcium: 9.13mg/dL, phosphorus:
1.17mg/dL, albumin 4.22g/dL, Hb: 13.0g/dL. His white blood cell
count and thrombocyte levels as well as liver function tests were
normal. Despite high glucose levels, he had neither acidosis nor
20 - 23 October, 2016
ketosis. He had overt hyperthyroidism with TSH: <0.05μIU/mL and
free T4: 3.47ng/dL. His thyroid receptor antibody level was found to
be positive (5.3U/L) as well as anti thyroid peroxidase antibody and
anti-thyroglobulin antibody levels. Thyroid ultrasonography was
consistent with thyroiditis. He was diagnosed as Graves’ disease
and TPP. Intravenous potassium supplementation was performed
with close monitoring and peroral 20mg propranolol as well as
30mg methimazole was given to the patient. His blood glucose levels were high being in the range of 250-400 mg/dl and his HbA1c
level was 4.7%. Basal-bolus insulin treatment was given during
his hospital stay and normoglycemia was achieved with insulin
treatment. Also his blood pressure was measured to be >140/90
mmHg during his stay and 10mg amlodipine was prescribed. After
normalization of potassium levels he didn’t have any complaints of
muscle weakness. His insulin requirement decreased during his
stay and he was discharged with 10 units of insulin glargine and
10mg amlodipine as well as 20mg propronolol and 30mg methimazole. He was invited to consultation 1 week after his discharge
and his free T4 level was 1.27ng/dL and he did not have any need of
insulin, amlodipine and propronolol.
Conclusion: TPP is an uncommon but potentially fatal complication of hyperthyroidism. Despite being an easily treatable condition,
it could have life-threatening consequences if it is overlooked.
Keywords: Thyrotoxicosis,
Graves’ disease
14
Antalya - Turkey
Transient
hypokalemic
paralysis,
2016
O - 07
Thyroid
A case with MEN-2b who has neurinoma of the
tongue and eyes
Hatice Özışık1, Banu Şarer Yürekli1, Nilüfer Özdemir Kutbay1, Mahir Akyıldız2,
Gökhan Özgen1
1 Department of Endocrinology, Ege University, İzmir, Turkey
2 Department of General Surgery, Ege University, İzmir, Turkey
Introduction: Multiple endocrine neoplasia type 2b (MEN 2b) is a
rare and dominantly inherited syndrome characterized by medullary thyroid cancer, pheochromocytoma and mucosal neuromas.
The most characteristic feature of MEN2b is multiple mucosal
neuromas seen in almost all cases. As it is rarely seen, we have
decided to present this case.
Case: A male patient aged 41 underwent bilateral total thyroidectomy operation due to multinodular goiter in 2004 and the pathological examination showed that the patient developed medullary thyroid carcinoma. In surveillance, upon the detection of pathological
lymph nodes in the neck, left neck dissection was applied.
In 2005, adjuvant radiotherapy was applied to the neck. In 2006,
in genetical analysis, M918T heterozygous mutation was detected
in RET gene. In 2008, as a mass was identified in the left adrenal
gland, laparoscopic left adrenalectomy was applied. The pathology
was determined as pheochromocytoma. Calcitonin value had not
been checked in the preoperative period. Calcitonin value was 120
pg/mL in the post-operative period.
Mutations at codon 883 and 918 mutations with MEN 2b are at increased risk of agressiveness. In our case,codon 918 mutation was
also detected.
It is recommended that the family members of these cases are
screened for medullary thyroid carcinoma and if there is any carrying these mutations, they should be treated with prophylactic total
thyroidectomy. For these cases, lifelong surveillance is required.
Keywords: Multiple endocrine neoplasia type 2b, medullary thyroid cancer, mucosal neuromas
Picture 1
In the family history of the patient, his elder brother had had an
operation due to pheochromocytoma.
In his physical examination, there were neuromas on the tongue
and eye lids (Picture 1). He had hyperpigmented areas on his left
shoulder (Picture 2)
Picture 1. Neuromas on the tongue
The latest value for calcitonin was 88.6 pg/mL and CEA was 6,78
ng/mL. 24 hour urine metanephrine and normetanephrine levels
were normal.
In laboratory examination, TSH was 2.26µIU/ mL and fT4 was 1.37
ng/dL. He was euthyroid with 150 mcg/day L-thyroxine treatment.
The patient has been surveilled through the ultrasonography of
neck (USG) and 24 hour urine metanephrine and normetanephrine.
Conclusion: The medullary thyroid carcinoma can progress most
agressively in MEN2b. In this syndrome metastasis were defined
even in the first year of life.
20 - 23 October, 2016
Picture 2. hyperpigmented areas on the left shoulder
15
Antalya - Turkey
2016
O – 08
Thyroid
A case of papillary thyroid carcinoma
in toxic adenoma: Are hyperfunctioning
nodules truly innocent all the times?
Banu Şarer Yürekli1, Hatice Özışık1, Nilüfer Özdemir Kutbay1, Özer Makay2,
Gökhan Özgen1, Ayşegul Akgün3
1 Ege University Faculty of Medicine, Endocrinology Department, izmir, turkey
2 Ege University Faculty of Medicine, General Surgery Department , izmir, turkey
3 Ege University Faculty of Medicine, Nuclear Medicine Department , izmir, turkey
Introduction: Thyroid scintigraphy should be performed for the
evaluation of thyroid nodules in case of suppressed Thyroid Stimulating Hormone (TSH) level. While the malignancy probability of
hypofunctioning nodules is 15%, this ratio is generally lower than
1% in hyperfunctioning nodules. So we would like to present a case
of toxic adenoma with the diagnosis of papillary thyroid carcinoma.
Case: Forty-four-year-old woman patient had presented with the
diagnosis of thyroid nodule. Thyroid fine needle aspiration biopsy
(TFNAB) was performed for the evaluation of thyroid nodule measured as 47x12 mm. This nodule was in mixed solid form bearing cystic components. TFNAB revealed that the thyroid nodule
was benign. Propylthiouracil had been started before admission
to our clinic according to laboratory values of TSH and free thyroxin (fT4) which were 0.005 µIU/ mL and 1.75 ng/dL, respectively. On admission to our endocrinology clinic we performed thyroid
scintigraphy showing hyperfunctioning thyroid nodule with scene
of suppressed rest of thyroid region. Thyroid auto-antibodies were
negative. Radioactive iodine therapy was recommended as a modality of treatment. However, the patient preferred surgery for her
20 - 23 October, 2016
treatment with the concern of her father’s death due to leukemia.
Our ultrasonographic evaluation was consistent with a thyroid
nodule located at right lobe with a diameter of 43x18x28 mm. The
nodule was mixed type solid nodule with regular borders and thin
hypoechoic peripheral halo. Central chaotic hypervascularity and
microcalcification were not detected. The patient underwent right
thyroid lobectomy. Pathology report revealed macrofollicular variant of papillary thyroid carcinoma. The tumor was 3.5 cm in diameter with regular border and 0.2 cm away from the surgical border.
The tumor didn’t spread out of the thyroid capsule. Second thyroid
surgery was performed due to remaining thyroid tissue. After that
ablative radioactive iodine therapy was applied.
Conclusion: Although malignant potential of hyperfunctioning
thyroid nodules is low, possibility of thyroid carcinoma cannot be
excluded in those thyroid nodules. So, hyperfunctioning thyroid
nodules warrants careful evaluation and appropriate therapy. We
wanted to draw attention of the clinicians for this rare issue.
Keywords: toxic adenoma, papillary thyroid carcinoma, surgery
16
Antalya - Turkey
2016
O – 09
Thyroid
Medically refractory amiodarone-induced
thyrotoxicosis: Two case reports
Narin Nasıroğlu İmga1, Zeynep Çetin1, Alper Çağrı Karcı1, Dilek Berker1,
Serdar Güler2
1 Department of Endocrinology, Ankara Numune Education and Research Hospital, Ankara, Turkey
2 Department of Endocrinology, Hitit University Faculty of Medicine, Corum, Turkey
Background: Amiodarone is the most commonly prescribed antiarrhythmic drug that is used mainly for the treatment of atrial
fibrillation. Amiodarone-induced thyrotoxicosis (AIT) is a therapeutic challenge and it may be due to iodine-induced excessive synthesis of thyroid hormone (Type 1) or a destructive thyroiditis (Type
2). Herein we present two cases of medically refractory AIT treated
with a surgical approach.
Case 1: A 24-year-old female with recurrent ventricular fibrillation
attacks due to cardiomyopathy was referred to our clinic because of
thyrotoxicosis. She had an intracardiac defibrillator (ICD), ejection
fraction (EF) was 35%, and had been using amiodarone orally for
three months. Thyroid function test (TFT) results revealed thyrotoxicosis. Thyroid gland size was normal but mildly parenchymal
heterogeneity and decreased vascularization were detected. Cardiology department discontinued the amiodarone therapy. Due to
diagnosis of type 2 AIT, steroid therapy was started. In addition to
increased steroid dosage, 3 weeks later methimazole and lithium
therapies were initiated because of the progression in clinical and
laboratory findings. Despite current medical therapies, no improvement was detected. Patient was diagnosed as non-responsive type
2 AIT and surgical approach was decided. In order to regulate the
TFT levels, 12 sessions of plasmapheresis were performed. Thyroid
function tests returned to normal. Subsequently, total thyroidectomy was performed without perioperative complication. Patient was
discharged from hospital by the third day.
20 - 23 October, 2016
Case 2: A 39-year-old male with history of hypertrophic cardiomyopathy due to ventricular arrhythmia, heart failure (EF 20%) and ICD
was hospitalized to the coronary intensive care unit. Amiodarone
infusion therapy was started. While amiodarone therapy continued
thyrotoxicosis was detected and patient was consulted to endocrinology clinic. The thyroid gland was normal but the vascularization
was reduced. Thyroid scintigraphy showed active involvement on
thyroid gland. Due to diagnosis of Type 1 AIT, antithyroid therapy
was initiated while streoid therapy was added a week later. The
therapy was continued in high doses but no response was seen and
arrhythmias continued. Because of high-risk for thyroid surgery
firstly 30 mCi radioiodine was administered but thyrotoxicosis continued. Therefore, total thyroidectomy was performed under strict
cardiology consultation. No perioperative complications were seen.
Levothyroxine replacement started and tapering of steroid therapy
was planned. Patient was discharged from hospital by the fourth
day.
Discussion: Amiodarone-induced thyrotoxicosis is a diagnostic challenge due to its complicated pathogenesis and unreliable
response to therapy. After the classification of AIT, the appropriate treatment must be started immediately. Persistent treatment
choices such as surgery should be considered in uncontrolled patients or patients that are refractory to medical treatment.
Keywords: Amiodarone, thyrotoxicosis, resistant
17
Antalya - Turkey
2016
O – 10
Lipids/Obesity
Metabolic abnormalities associated with
insulin resistance in a young patient with
acquired partial lipodystrophy
Nilüfer Özdemir Kutbay1, Banu Sarer Yürekli2, Halit Diri1, Hatice Özışık2, Zeki Yaşar3,
Füsun Saygılı2, Barış Akıncı4
1 Gazi Yasargil Training and Research Hospital, Division of Endocrinology, Diyarbakir, Turkey
2 Ege University, Division of Endocrinology, Izmir, Turkey
3 Selahaddin Eyyubi State Hospital, Division of Plastic Surgery, Diyarbakir, Turkey
4 Dokuz Eylul University, Division of Endocrinology, Izmir, Turkey
Introduction: About 250 patients with acquired partial lipodystrophy (APL) have been reported so far. It is characterized by the loss of
adipose tissue from the face, arms and upper part of the body. The
disease usually starts during childhood or adolescence. Fat loss
often come into view in months or years. Females are more frequently affected. Although the etiology of APL is unclear, increased
complement activity has been reported in some APL patients.
Conclusion: Although previous studies reported that metabolic abnormalities were rarely observed in APL, our recent study showed
that metabolic abnormalities associated with insulin resistance
are more common than previously thought. Our observation on this
newly diagnosed APL patient also supports the idea that a regular
screening for metabolic abnormalities should be carried out in patients with APL even at young age.
Case: A 23-year-old female reported fat loss on her face that she
first noticed when she was 12. She was diagnosed with diabetes
when she was 21 which was treated with metformin. On physical
examination, fat loss was remarkable on her face, arms and upper trunk (Fig.1a). Acanthosis nigricans was noticed on her armpits and around her neck (Fig.1b). Her laboratory results were as
follows: fasting blood glucose: 111 mg/dl, HbA1c: 6.1%, fasting insulin: 34.3 ulU/ml, AST: 40 U/L, ALT: 65 U/L, triglyceride: 393 mg/
dl, HDL-cholesterol: 32 mg/dl, C3: 1.02 g/L (0.9-1.8), C4:0.29 g/L
(0.1-0.4), and microalbumin in spot urine: 26.5 mg/L. Abdominal
ultrasonography showed grade 1 hepatosteatosis. Echocardiography was normal. No Drusen-like lesion was detected in eye examination.
Keywords: Acquired partial lipodystrophy, metabolic abnormalities,Drusen-like lesion
Figure 1a, 1b: A case of acquired partial lipodsyttrophy (APL)
20 - 23 October, 2016
18
Antalya - Turkey
2016
O - 12
Bone/Calcium
Effect of zolendranate treatment on
hearing loss in a patient with the Paget’s
disease who has skull involvement
İlkay Kartal1, Arzu Tatlıpınar2
1 Department of Internal Medicine, Division of Endocrinology and Metabolism, Medeniyet University, Goztepe
Training and Research Hospital, istanbul, turkey
2 Department of Otolaryngology- Head and Neck Surgery, Fatih Sultan Mehmet Training and Research Hospital,
istanbul, turkey
Background: Paget’s disease is a localised disorder of bone remodelling characterized by the excessive resorption and new bone
formation. Clinical features of Paget’s disease are pain, fractures,
deformity, and manifestations of the neurologic, rheumatologic, or
metabolic complication of the disease. However, at least two-thirds
of patients are asymptomatic.
The patient was sent to ear specialist for evaulation of her hearing
loss. Physical examination of her ears were normal. In her audiometric assesment there was bilateral asymmetric sensorineural
hearing loss in pure tone audiometry of patient. Pure tone averages
of air/bone conduction were 25 dB/25 dB on the right side and 35
dB/27 dB on left side.
Case: We reported a 50 years old woman attend neurosurgery outpatient clinic complaining of headache for about a year. The patient was
reffered to endocrinology outpatient clinic because the cranial magnetic resonance imaging (MRI) of the patient raised suspicion of Paget’s disease of bone. From the anamnesis of the patient we learned
that she also has progressive hearing loss. On physical examination,
she has enlargement of the skull with left frontal bossing.
After vitamin D deficiency was corrected serum PTH level became
normal but serum ALP level was still high. A single 5 mg dose iv
zolendronate was given for the treatment of her Paget’s disease.
Six months following this treatment serum ALP activity, calcium,
phosphorous, PTH levels are in normal limits. At the audiometric
reassesment pure tone audiometry averages of air/bone conduction are 22 dB/17 dB on the right side and 37 dB/32 dB on the left
side. Her hearing loss has not changed significantly. The differences between pure tone averages of two periods are minimal and can
be audiometrist dependent.
Laboratory findings; serum calcium, phosphorus, urea, creatinine,
liver enzyme levels were normal. Serum alkaline phosphatase
(ALP) activity and parathyroid hormone (PTH) levels were increased
and serum vitamin D level was deficient.
Imaging studies; Cranial MRI of the head show marked thickening of
the diplopic space, diffuse calvarial thickening. Left side is more thick
than the right side. There are heterogenous signal differences in all
sequences. Bone scan releaved intense uptake of the radionuclide in
the calvarium that is generally diffuse but heterogenous only in some
parts and more marked in the left fronto-parietal bone. There is also
intense uptake of the radionuclide on the left femur.
20 - 23 October, 2016
Conclusion: Hearing loss due to Paget’s disease may not be reversible but early diagnosis and treatment may prevent further
deterioration of hearing. Randomized, double-blind, placebo controlled clinical trials in which the effect of antipagetic therapy is
assessed on hearing loss in patients with Paget’s disease of the
temporal bone, are needed.
Keywords: hearing loss, paget’s disease, bisphosphonate
19
Antalya - Turkey
2016
O – 13
Bone/Calcium
Thyroid nodule or parathyroid adenoma: a
case report
Eda Çalışkan Yıldırım, Uğur Ünlütürk, Nafiye Helvacı, Miyase Bayraktar
Division of Endocrinology and Metabolism, Department of Internal Medicine, Hacettepe University, Ankara,
Turkey
Introduction: Primary hyperparathyroidism is a disorder caused
by enlargement of one or more parathyroid glands. About 6-16%
of parathyroid adenomas can be in an ectopic location, therefore,
localization of parathyroid lesions can be challenging.
Case Report: A 74-year-old female patient was admitted to our
hospital with weakness, fatigue and constipation. Her medical
history included type 2 diabetes mellitus, hypertension and coronary heart disease. Laboratory studies revealed normal iPTH concentration (56.9 pg/mL normal: 12-88 pg/mL) and normal serum
biochemistry except elevated calcium level (13.15 mg/dL). Urinary
calcium excretion was to be high (549.15mg/day). Based on clinical
and laboratory findings, a final clinical diagnosis of primary hyperparathyroidism was made.
Neck ultrasonography exam was planned and performed with the
intention to localize the adenoma. A 14x10 mm, hyopoechoic solid
lesion was located in the inferior pole of the right thyroid parenchyma. In addition to this nodule there were also several other subcentimeter solid nodules scattered throughout both thyroid lobes.
The hypoechoic solid nodules described in the inferior pole of the
right thyroid lobe was biopsied with fine needle aspiration (FNA)
under US guidance. The cytological exam revealed atypia of un-
20 - 23 October, 2016
determined significance. The following 99mTc-MIBI scintigraphy,
SPECT–CT, neck MRI and 4-D neck CT did not depict any findings
suggestive of a parathyroid adenoma. Under the light of these findings we considered that an intrathyroidal parathyroid adenoma was
clinically possible and a second FNA biopsy with PTH washout was
performed. PTH washout concentration was found to be conclusive
for a parathyroid adenoma with the level of 2612 pg/mL.
Based on these findings surgical removal of the right thyroid lobe
was planned. Intraoperative frozen exam revealed this nodule to
represent a papillary thyroid carcinoma and a total thyroidectomy
was performed after this result. Post-surgical definitive pathological exam confirmed that this nodule was consistent with a parathyroid adenoma instead of PTC. Post-operative serum PTH and
calcium levels returned to normal values.
Discussion: Intrathyroidal parathyroid adenomas may cytologically
mimic primary papillary thyroid carcinomas. In this case report, we
aimed to underline the diagnostic value of PTH washout after FNA
for suspicious intrathyroidal nodules.
Keywords: parathyroid adenoma, hypercalcemia, fine needle aspiration, parathyroid hormone
20
Antalya - Turkey
2016
O - 14
Thyroid
A case of thrombotic thrombocytopenic
purpura in a toxic multinodular goiter
patient on antithyroid medication
Gökhan Tazegül1, Tahir Saygın Öğüt1, Hümeyra Bozoğlan2, Özlem Doğan2,
Nusret Yılmaz2, Ozan Salim3, Ramazan Sarı2, Hasan Ali Altunbaş2, Mustafa Kemal Balcı2
1 Akdeniz University, Faculty of Medicine, Department of Internal Medicine, Antalya/Turkey
2 Akdeniz University School of Medicine, Department of Endocrinology, Antalya/Turkey
3 Akdeniz University School of Medicine, Department of Hematology, Antalya/Turkey
Introduction: Thrombotic thrombocytopenic purpura (TTP) is a
rare disease characterized by thromboses, resulting in thrombocytopenia. It consists of the pentad of microangiopathic hemolytic
anemia, thrombocytopenic purpura, neurologic abnormalities, fever, and renal disease. It’s unclear what triggers TTP, but some
factors may play a role such as: pregnancy, malignancies, infections, surgery, blood and marrow stem cell transplant, chemotherapeutics, ticlopidine, clopidogrel, cyclosporine A, estrogens and
quinine. The association between hyperthyroidism or antithyroid
drugs with TTP is nonexistent in the literature; this case is the first
in the literature to discuss possible hyperthyroidism or antithyroid
medication triggered TTP.
Case: A fifty four years old female patient was diagnosed with toxic
multinodular goiter and treated with propylthiouracil for two weeks
and methimazole for a week before admission. Patient was admitted to emergency room with disseminated purpura, acute renal
failure and lethargy. Her routine blood work revealed elevated creatinine [4.8 mg/dL (0.5-1.1 mg/dL)] and thrombocytopenia [13.000/
mm3 (150.000-450.000/mm3)]. Coagulation tests were normal. Peripheral blood smear revealed shistocytes; patient was diagnosed
as TTP and was hospitalized by Hematology. TSH levels were low
[<0.01 mIU/mL (0.35-5.5 mIU/mL)] and free T4 and T3 levels were
20 - 23 October, 2016
elevated [T4: 3.28 ng/dL (0.72-1.56 ng/dL), T3: 16.3 pg/mL (1.84.6 pg/mL)]. ADAMTS13 levels were low; ADAMTS13 inhibitor was
high. During her treatment for TTP, she was consulted with Endocrinology for hyperthyroidism. She did not have any symptoms
related to hyperthyroidism; hematology planned plasmapheresis
twice daily and corticosteroid treatment for TTP. We did not plan
any additional urgent treatment for hyperthyroidism, we decided
to follow up the patient with plasmapheresis since antithyroid drug
related TTP was a possibility. After the patient was in remission
from TTP, plasmapheresis was stopped, we planned radioactive
iodine treatment. She was discharged without any complaints. At
3 months follow-up, she was in remission for TTP and her thyroid
function tests showed subclinical hyperthyroidism.
Discussion: Here we present a case of TTP possibly triggered either by hyperthyroidism or antithyroid drugs. Laboratory results
show that TTP is acquired in this patient. It is not possible to differentiate between drug-induced or disease-induced TTP. Since
drug-induced case is a possibility, we believe that it was relatively
not safe to use antithyroid medication in this patient.
Keywords: toxic multinodular goiter, metimazole, thrombotic
thrombocytopenic purpura
21
Antalya - Turkey
2016
O - 15
Thyroid
Agressive Follicular Variant of Papillary
Microcarcinoma Presenting with Extensive
Bone Metastases and Associated with Weird
Thyroid Function Tests
Nurdan Gül1, Hülya Hacışahinoğulları1, Metban Mestanzade1, Ayşe Kubat Üzüm1,
Özlem Soyluk Selçukbiricik1, Sevgi Kalayoğlu Beşışık2, Refik Tanakol1
1 Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Endocrinology and Metabolism ,
istanbul, turkey
2 Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Hematology , istanbul, turkey
Introduction: Papillary microcarcinomas can rarely manifest as
aggressive bone metastatic lesions with unexpected laboratory and
clinical findings. We herein present an elderly patient diagnosed
with multiple bone metastasis during investigations for acute kidney injury.
Clinical Case: In February 2015, a 72-year-old woman experienced
a car accident, which lead to right humerus fracture and acute kidney injury. Her previous history was remarkable for tuberculosis,
hydatid cyst, type 2 diabetes and hypertension, and she donated
her kidney to her daughter. She also suffered from widespread
bone pain for 1 year, and abdominal CT revealed multiple bone
lesions involving lumbar spine and ischial bone. Pelvic MRI and
bone scan showed extensive lytic bone lesions involving mandible,
humerus, femur, multiple ribs, manubrium sterni, scapula, clavicle, lumbar and sacral spine and pelvic bones. Bone biopsy was
compatible with thyroid cancer. Thyroid ultrasound revealed multiple nodules, and biopsy was suspicious for follicular neoplasm.
The patient underwent total thyroidectomy and neck dissection,
and histopathological investigations showed two foci of follicular
variant of papillary microcarcinoma in the right lobe. External radiotheraphy was also performed for some of the metastatic bone
lesions. Postoperatively, she was treated with 200 mCi 131I-therapy combined with temporary high dose dexamethasone. Whole
body scan after treatment showed uptakes in the neck and bones.
Levothyroxine was begun and the dose was increased to 150 mcg/
day, but the patient began to experience dyspnea and pretibial ede-
20 - 23 October, 2016
ma due to diastolic congestive heart failure. Thyroid function tests
showed a very low fT4, high fT3 and supressed serum TSH. During
follow-up L-thyroxine dose was tapered to 50 mcg/day. Her weird
thyroid function tests were possibly due to tumor tissue expressing
excessive type 2 deiodinase activity converting circulating T4 to T3.
She did not have functioning thyroid cancer metastases, since the
patient’s thyroid hormone levels fell when levothyroxine supplementation was held, and her TSH levels were increased. After the
first radioactive iodine therapy she experienced progressive tumor
burden, and another 200 mCi radioactive iodine was administered,
which resulted in tumor lysis syndrome, DIC, bone marrow failure,
and she needed hemodialysis. She rejected further treatment and
died of extensive metastatic disease at home.
Conclusion: Although papillary microcarcinomas are known to be
tumors with good prognosis, they can also present with extensive
refractory and fatal bone metastasis. Some of the weird thyroid
function tests such as low free T4 and normal to high free T3 levels
and comparably low levothyroxine need to supress TSH may be associated with type 2 deiodinase activity in the tumor. In these conditions, increasing levothyroxine dosage for normalising free T4 level
is useless and may even lead to very high T3 levels and associated
adverse affects.
Keywords: follicular variant of papillary microcarcinoma, bone
metastasis, thyroid function tests
22
Antalya - Turkey
2016
O - 16
Thyroid
Thyrotoxic Periodic Paralysis
Ethem Turgay Cerit1, Mehmet Tuncay2, Mehmet Muhittin Yalçın1
1 Department of Endocrinology & Metabolism, Dr. Ersin Arslan Training and Research Hospital, Gaziantep,
Turkey
2 Department of Nephrology, Dr. Ersin Arslan Training and Research Hospital, Gaziantep, Turkey
Introduction: Thyrotoxic periodic paralysis (TPP) is characterized
by sudden onset of hypokalemia and paralysis under the condition
of thyrotoxicosis. TPP is more prevalent in Asians than in nonAsians and also in males more than in females. In Chinese and
Japanese patients with hyperthyroidism, 1.8–1.9% experience TPP.
The typical age of onset is 20–40. Hypokalemia in TPP results from
an intracellular shift of potassium under conditions of high thyroid
hormone levels.
Clinical Case: A 29-year-old man with a history of recurrent muscle weakness presented to our emergency department with difficulty in walking. The patient’s symptoms started one day before,
and he was unable to walk to the hospital. He had had similar episodes before. He stated palpitations, tremor and excessive sweating. He had no family history of periodic paralysis.
On physical examination, he had an enlarged thyroid gland and
tachycardia. He had tremor in his hands and decreased muscle strength in both lower extremities. His initial potassium level was 2.6 mEq/L (3.5–5.1 mEq/L), phosphorus level was 3.7 mg/
dL (2.5–4.5 mg/dL), and magnesium level was 1.9 mg/dL (1.7–2.6
mg/dL). Electrocardiogram showed sinus tachycardia with a rate
of 124 beats per minute. He was given intravenous potassium
chloride with saline infusion. He was also given oral potassium
replacement, which resulted in resolution of his lower-extremity
paralysis. On the second day of admission, his serum potassium
level increased to 4.8 mEq/L. His renin and aldosterone levels were
20 - 23 October, 2016
within normal limits. The thyroid-stimulating hormone (TSH) level
was 0.01 mU/L (0.4–3.9 µIU/mL), the free thyroxine (fT4) level was
3.8 ng/dL (0.8–2.7 ng/dL) and the free triiodothyronine (fT3) level
was 13.8 ng/dL (2.3–4.4 pg/mL). Thyrotoxicosis was diagnosed, and
the patient was given propranolol 40 mg twice a day. The anti-TSH
receptor antibody level was 19.6 IU/L (1.22-1.75 IU/L), His thyroid
scintigraphy and uptake scan was consistent with Graves’ disease.
He was started on methimazole 20 mg per day. Serial measurements of his serum potassium level remained within normal limits
without potassium replacement. The patient was diagnosed as TPP
secondary to Graves’ disease.
CONCLUSION: The diagnosis of TPP is made when a patient presents with a paralytic attack that is associated with hypokalemia and
hyperthyroidism. The most common underlying form of thyroid disease associated with TPP is Graves’ disease but any other cause
of hyperthyroidism can be associated with TPP. The condition may
be life-threatening if weakness of the breathing muscles leads to
respiratory failure, or if the low potassium levels lead to cardiac
arrhythmias. Patients should be on cardiac monitoring, and potassium levels should be monitored for potential rebound hyperkalemia. Treatment of TPP includes replacing potassium, prevention
of this shift of potassium by using nonselective beta-blockade and
treatment of underlying thyrotoxicosis.
Keywords: thyrotoxicosis, hypokalemia, paralysis
23
Antalya - Turkey
2016
O - 17
Thyroid
Thyrotoxicosis with pleural effusion and
hypoalbuminemia
İffet Dağdelen Duran1, Neşe Ersöz Gülçelik2
1 Department of Endocrinology and Metabolic Diseases, Kırıkkale Yüksek İhtisas Hospital, Kırıkkale, Turkey
2 Department of Endocrinology and Metabolic Diseases, Ankara Education and Research Hospital,
Ankara,Turkey
Introduction: Hypothyroidism and pleural effusion (PE) are
well-defined to accompany, however thyrotoxicosis and PE collaboration is not so frequent. We described a patient with no history
of heart or liver failure; suffering from PE, peripheral edema, and
hypoalbuminemia who was diagnosed with thyroxicosis. PE and
peripheral edema resolved immediately after initiation of antithyroid medication.
Case- A 50-year old man arrived at our clinic with fatique, shortness of breath, palpitations and edema in lower extremity. He had
a pulse rate of 130/min with a respiratory rate of 28/min, while
pulmonary examination defined decreased sounds in right inferior and mid lobe and there was grade 3 pretibial edema. His blood
test results were: Creatinine: 0,32 mg/dl, Albumin(alb): 2.8 g/dl,
Alanine Aminotransferase (ALT): 21 U/l, Gamma Glutamyl Transferase (GGT): 48 U/l, Thyroid Stimulating Hormone (TSH): 0.00 µIU/
ml, free T4 (fT4): 3. 6 (0.89-1.79) ng/dl, free T3 (fT3): 8.7 (2.3-4.2)
pg/ml. His chest graph revealed right pleural effusion and chest
computed tomography (CT) showed 27 mm pleural effusion in
right hemithorax. Echocardiography (echo) demonstrated grade 2
mitral regurgitation (MR), tricuspid regurgitation (TR) and normal
ejection fraction. Hepatobiliary ultrasonography (USG) showed no
pathology. Spironolactone 25 mg/d, hydrochlorthiazide 25 mg/d
and furosemide 40 mg/d was started. Thyroid USG revealed multinodular goiter, the biggest nodule 18x22x34mm on right lobe and
thyroid 99m Technetium-pertecnatate scintigraphy showed diffuse
20 - 23 October, 2016
increased uptake and a hypoactive nodule in right lobe. After toxic
diffuse multinodular goitre (TDMNG) was diagnosed, MMI 30 mg/
day was attempted to start. Since unavailability of MMI, PTU 300
mg/day was started. After one week, fT4: 2.55 ng/dl, fT3: 8.37 pg/
ml were found along with increase of alb to 3.5 g/dl. Meanwhile
pulmonary sounds reversed to normal and chest graph revealed
normal examination with peripheral edema resolved in lower extremity. Echo showed trace amount of TR and MR. After one month
TSH:0.00 µIU/ml, fT4: 2.24 ng/dl, fT3: 7.95 pg/ml and 3.8 g/dl alb
level were seen. A month later, alb level rised to 4.6 g/dl.
Conclusion: PE is rare, while hypoalbuminemia is again unexplained in thyrotoxic patients. Thyrotoxic state establishes inflammation that would cause alb to decrease. In our case hypoalbuminemia evolved to be noteworthy since it would describe PE and
peripheral edema accompanying thyrotoxicosis. After antithyroid
treatment, volume excess resolved with alb rise. Nevertheless,
multiple diuretic medications may also improve the patients’ ailments. Diuretics may have decreased the volume overload and mediate alb level rise by increasing alb concentration. However abrupt
disappearance of such volume load would not be depended only on
low dose oral diuretic treatment. PE may be seen in patients with
thyrotoxicosis and responds well to antithyroid therapy and low
dose diuretic therapy.
Keywords: Thyrotoxicosis, pleural effusion, hypoalbuminemia
24
Antalya - Turkey
2016
O - 18
Thyroid
Plasmapheresis As A Temporary Management
Option In A Patient With Graves’ Disease and
Primary Sclerosing Cholangitis
Batuhan Başpınar1, Seda Hanife Oğuz2, Taylan Kav3, Bülent Okan Yıldız2
1 Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, Turkey
2 Division of Endocrinology and Metabolism, Department of Internal Medicine, Hacettepe University School of
Medicine, Ankara, Turkey
3 Division of Gastroenterology, Department of Internal Medicine, Hacettepe University School of Medicine,
Ankara, Turkey
Introduction: Graves’ disease is an autoimmune disorder which
can be accompanied with other autoimmune diseases affecting
various organ systems. In such cases, treatment of hyperthyroidism may be troublesome due to organ dysfunction caused by concomitant disease. Herein, we present a patient with liver failure due
to primary sclerosing cholangitis complicated with Graves’ disease.
Case Presentation: A 35-year-old man, who was diagnosed with
Friedreich Ataxia at the age of 12 and primary sclerosing cholangitis two months ago, presented to our gastroenterology clinic with the
complaints of pruritus, diarrhea, palpitations and weight loss. He was
currently taking ursodeoxycholic acid and proton pump inhibitor therapy. On physical examination, he had jaundice, ataxic gait, choreiform
movements. Laboratory workup yielded elevated ammonia levels
indicating hepatic encephalopathy in addition to very high bilirubin,
transaminase and prothrombin time levels. Thyroid function tests
suggested primary hyperthyroidism. TSH receptor antibody (TRAb)
was positive (6.27 IU/L). Thyroid ultrasonography showed parenchymal heterogeneity in a normal sized gland, which was diffusely hyper-
20 - 23 October, 2016
active in technetium 99m pertechnetate scintigraphy. The patient was
diagnosed as Graves’ disease. Antithyroid druq therapy was not suitable for the patient because of liver failure. Therefore, the patient underwent plasmapheresis as a temporary management option before
definitive treatment for thyrotoxicosis. After two sessions of plasmapheresis, TRAb became negative, fT4 and fT3 levels decreased, clinical
findings of hepatic encephalopathy improved due to decreasing bilirubin levels and the need for urgent liver transplantation disappeared.
After discharge, total thyroidectomy was performed. Pathological examination revealed diffuse hyperplasia and multifocal thyroid micropapillary carcinoma.
Conclusion: Plasmapheresis is a viable option for patients with
Graves’ disease and impaired liver function. In our case, only two
sessions of plasmapheresis enabled the patient to have thyroid
surgery and resolved the need for urgent liver transplantation.
Keywords: Plasmapheresis, Graves’ disease, primary sclerosing
cholangitis
25
Antalya - Turkey
2016
O – 19
Thyroid
A lump on the scalp: An unusual presentation
of papillary thyroid carcinoma
Damla Eyüpoğlu1, Uğur Ünlütürk2
1 Department of Internal Medicine, Hacettepe University, Ankara, Turkey
2 Department of Endocrinology and Metabolism, Hacettepe University, Ankara, Turkey
Introduction: Papillary thyroid carcinoma (PTC) is the most common malignancy of the thyroid gland. Metastasis to the regional
lymph nodes is relatively high, and the most common distant metastasis sites are the lung and bone. Skin metastasis of PTC is very
rare, whereas scalp is the most frequent cutaneous area involved.
Case Presentation: 80-year-old female with history of hypertension and diabetes was referred to neurosurgery clinic due to a progressively enlarging lump at the frontal head region for 4 years.
CT scan revealed a heterogeneously enhancing semisolid lesion
measuring 115x84x69 mm. The lesion was found to be causing extensive frontal bone destruction with associated superior sagittal
sinus invasion. Incisional biopsy of the lesion came back as welldifferentiated thyroid carcinoma metastasis with positive staining
for TTF-1 and thyroglobulin. Her TSH level was suppressed with
a value of 0.18 mIU/ml and with normal fT4 and fT3 levels (10.56
pmol/L, fT3: 5.94 pmol/L, respectively). Thyroid ultrasonography
exam revealed a left lobe located hypoechoic solid nodule with peripheral coarse eggshell calcifications measuring 30x20 mm. This
nodule was found to be cold on thyroid scintigraphy. US guided fine
needle aspiration biopsy of the nodule was reported to be highly
20 - 23 October, 2016
suspicious for follicular thyroid carcinoma. Genomic studies revealed negative Pax8-PPARg gene rearrangement. PET-CT study
performed after the biopsy did not detect any additional metastatic
foci. The patient underwent total thyroidectomy and final pathologic examination revealed a 4 cm diameter and encapsulated type
papillary thyroid carcinoma of follicular variant. The serum fT4 and
fT3 levels remained elevated after total thyroidectomy (with the levels of 37.78 pmol/L and 12.04 pmol/L, respectively) a finding which
we ascribed to continued, and uncontrolled, thyroid hormone production by the metastatic head lesion.
Based on these clinical and laboratory findings, we started oral
methimazole therapy. After consultation with neurosurgery department the lesion was planned for cytoreductive surgery as the
patient was not a candidate for complete resection.
Discussion: Cutaneous thyroid carcinoma metastasis is highly
unusual and it generally occurs in the setting of disseminated advanced stage disease.
Keywords: papillary thyroid carcinoma, skin metastasis,scalp
26
Antalya - Turkey
2016
O – 20
Lipids/Obesity
Wernicke encephalopathy in a case who had
gastric bypass surgery due to morbid obesity
Hatice Özışık1, Banu Şarer Yürekli1, Nilüfer Özdemir Kutbay1, Ilgın Yıldırım Şimşir1,
Can Emre Erdoğan2, Füsun Saygılı1
1 Department of Endocrinology, Ege University, İzmir, Turkey
2 Department of General Neurology, Ege University, İzmir, Turkey
Introduction: Wernicke encephalopathy (WE) is an acute neurologic
disorder and results from deficiency of thiamine (vitamin B1). Traditional clinical triad is occulomotor abnormalities, confusion and gait
ataxia. It has a high chance of recovery through thiamine treatment.
However, since there is a serious mortality risk, conditions other than
alcoholism which may lead to malabsorption should be included in the
differential diagnosis. In this report, a WE case which developed after
Roux-en-Y gastric bypass surgery due to morbid obesity is presented.
is a disorder resulting from a combination of ataxia, ophthalmoplegia, nystagmus, mental confusion and lack of vitamin B1. Thiamine
deficiency can be seen in alcoholism, prolonged fasting, hyperemesis
gravidarum, prolonged parenteral nutrition, dialysis and AIDS. If Wernicke Encephalopathy is not treated, it can cause catastrophic amnesia, Korsakoff’s psychosis and death. In conclusion, like in this case, it
should be kept in mind that Wernicke Encephalopathy may be seen in
patients who have complaints of dizziness after gastric bypass.
Case Report: A female patient, aged 47 and known to have had diabetes mellitus for 17 years and getting insulin treatment, underwent a
gastric bypass operation in November 2014 since BMI was 51.4 kg/m2.
After two months from the operation, the patient was hospitalized due
to dizziness, fatique, nausea and vomiting. After 6 months from the
operation, the patient was observed to have lost 53 kg.
Keywords: Wernicke encephalopaty, thiamine, gastric bypass
In the physical examination, the general condition of the patient
was good; and there were pearlescent striae and surgical scars on
the abdomen. In the neurological examination, the patient was conscious, cooperative, oriented and had end-point nystagmus. Deep
tendon reflexes were symmetrical and normal, romberg test was
negative but ataxia was present.
In biochemical examination, Hba1c was %7.6, Hb was 9.7 g/dl, creatinine was 1.12 mg/dL, and iron parameters, B12 and folic acid
were normal. In cranial MRI, multiplane monitoring was carried
out at TSE (turbo spin echo) and FLAIR (fluid-attenuated inversion
recovery) sequences. In supratentorial sections, on T2-weighted
and FLAIR images hyperintensity was detected in mamillary body
and periaqueaductal area. In diffusion weighted MRI, acute ischemia or diffusional restriction were not detected. With reference
to the MRI findings, WE was thought to be present in the patient
who had rapid weight loss after gastric bypass surgery. Intravenous
thiamine treatment 500 mg/day was started. It was given for two
days and then the treatment continued with oral administration of
vitamine B. The symptoms were regressed significantly in a week.
Conclusion: Roux-en-Y gastric bypass is a bariatric surgery which
gives good results in the long term. However, in the post-operative period nausea and vomiting occur frequently. Wernicke Encephalopathy
20 - 23 October, 2016
Figure 1. Periaquaductal involvement in axial T2
Figure 2
Figure 2. Mamillary body in T2 FLAIR(marked with pink arrow) and
periaquaductal hyperintense areas(marked with yellow arrow)
27
Antalya - Turkey
2016
O - 21
Diabetes
The investigation for celiac disease in a
newly diagnosed patient with diabetes is
necessary in any case
Maria Papagianni1, George Paltoglou2, George Mastorakos3
1 Endocrine Unit, Third Department of Pediatrics, Aristotle University of Thessaloniki, Hippokrateion General
Hospital of Thessaloniki, Thessaloniki, Greece
2 First Department of Pediatrics, “Aghia Sofia” Children’s Hospital, Athens University Medical School, Athens,
Greece
3
Endocrine Unit, Second Department of Obstetrics and Gynecology, Areteion Hospital, Athens University
Medical School, Athens, Greece
Background: Celiac disease (CD) is a malabsorption syndrome. Its
coexistence with type 1 diabetes mellitus (t1DM) has been proven.
Growth retardation is the most common cause that leads to the
diagnosis in childhood and adolescence.
Clinical Case: A 7.5-year-old girl presented with hematuria and polyuria. She was born at term after an uneventful pregnancy with normal
vaginal delivery (birth weight: 3720gr). Her height followed the 97th
percentile (her target height was at 75th percentile). Since then, her
weight increased inappropriately due to unhealthy diet and exercise
absence. At the admission her BMI was 24.4 (>97th centile). She developed body odor and pubic hair at the age of 7 years. Both her parents
had constitutional delay of growth and puberty. Regarding her family
history, her mother and maternal grandmother had nephrolithiasis,
her maternal grandfather had t2DM and her father had hypertension.
Her mother also reported a history of gestational diabetes during her
second pregnancy. On physical examination, she was prepubertal
(Tanner stage: Breast 1, Pubic Hair 2, Axillary Hair 1) and acanthosis
nigricans was noticed. Her initial investigation tests revealed cystitis
and severe hypeglycemia. Her HbA1c level was 9.1% and she was put
on insulin treatment and antibiotics. Due to obesity and family history
of diabetes, it was ordered further laboratory workup that supported
20 - 23 October, 2016
the diagnosis of t1DM by revealing very high Anti-GAD and Anti-insulin antibodies levels. Moreover, she was checked for Hashimoto thyroiditis and CD and was found very high Antigliadin IgG, Anti-tTG IgA
and Anti-endomysial antibodies levels. HLA class II typing revealed
the presence of DQA1*05 and DQB1*02 alleles that encode HLA-DQ2
heterodimer found in approximately 95% of patients with CD. Subsequently, gastroscopy led to the diagnosis of CD. Her bone age was 2.5
years ahead of chronological age. She was prescribed a specific diet
and exercise program that led to weight loss while two months later,
following the CD diagnosis, she was put on gluten free diet. As soon
as she left hospital, her insulin requirements decreased and 20 days
after the onset of therapy she stopped insulin due to repeated hypoglycemic episodes. Her HbA1c level was within normal range 4 months
later and she was off insulin treatment for 8 months. After that period
hyperglycemic episodes were noticed, HbA1c level increased and she
was put on insulin treatment again.
Conclusion: Even though obesity is very uncommon between patients with CD, it is necessary for every patient with t1DM to be
evaluated for CD at the time of t1DM diagnosis and on regular basis
thereafter.
Keywords: diabetes, celiac disease, obesity
28
Antalya - Turkey
2016
O – 22
Diabetes
Mauriac syndrome still exists; a rare
complication of type 1 diabetes mellitus
Anara Karaca, Elif Topçuoğlu, Neşe Ersöz Gülçelik, Tülay Omma, Nujen Çolak Bozkurt,
Sevde Nur Fırat, Tahsin Özenmiş, Gönül Koç, Cavit Culha, Yalçın Aral
Department of Endocrinology and Metabolism, Ankara Teaching and Research Hospital, Ankara, TURKEY
Mauriac syndrome (MS) is an underrecognized complication, characterized by short stature, delayed puberty mostly in adolescents
with poor control of type 1 diabetes metllitus (T1DM). It presents
with cushingoid features and hepatomegaly due to hepatic glucogenosis with altered liver enzymes.
A 20-year-old female with a-17 year history of T1DM was admitted with diabetic ketoacidosis and for further evaluation of her latest poor glycemic control and abdominal distension, she had for a
long time. The patient was on intensive insulin treatment since her
disease onset. Her first diabetic ketoacidosis was documented in
2014. Her present admission was due to her third diabetic ketoacidosis. Her ever lowest HbA1c was 8%, but last several years her
A1c levels have been measured between 9.2-11%.
Anthropometric data revealed height 146 cm (<3 rd percentile),
weight 44 kg (<3 rd percentile), body mass index 20.6 kg/m2. The
patient menarch age was 18 and she was pubertal with Tanner
stage III for breast and pubic hair development.
She complained about the distention and tenderness due to her protuberant abdomen, and physical examination revealed tender and
4-5cm enlarged liver below her rib cage. Additionally, she had Cushingoid features; moon face, high pitched voice and abdominal obesity.
charge were: ALT:75, AST:61. A1c:8.2%.
MS is a syndrome of growth failure and delayed puberty associated
with massive liver enlargement from glycogen deposition in patients with poorly controlled T1DM, which is rarely seen nowadays.
Liver biopsy should confirm the hepatic glucogen deposition.
A mechanistic cause for this syndrome is unknown. However, this
year a mutation in the catalytic subunit of liver glycogen phosphorylase kinase was discovered by MacDonald et al. The effect of a mutant
enzyme of glycogen metabolism can combine with hyperglycemia to
directly hyperinhibit glycogen phosphorylase, in turn blocking glycogenolysis causing the massive liver in Mauriac disease.
The main treatment of Mauriac syndrome is the amelioration of
glycemic control that is usually accompanied by the reversal of the
liver disorder.
The result of genetic test of this patient is pending.
Keywords: type 1 diabetes mellitus, mauriac syndrome, hepatic
glycogen deposition
Dynamic liver MRI, due to her distention and tender liver, demonstrated enlarged right lobe of liver of 17cm size, left lobe reaching
and covering across her spleen.
Lab Results: ALT: 101 (0-35), AST: 83( 0-35), A1c:9.4%, fasting
plasma glucose:374. Liver biopsy revealed swollen hepatocytes
and glycogenic hepatopathy, which ensured, a rare complication of
T1DM, the Mauriac syndrome.
Evaluation for microvascular complications demonstrated no diabetic retinopathy and nephropathy. Besides, no diabetic neuropathy
was detected. The average blood pressure was 110/65mmHg.
After glycemic control was established, her distention and liver
tenderness were regressed. As a result of phycological assesment
treatment due to anxiety was initiated. Liver enzymes before dis-
20 - 23 October, 2016
Figure 1: Dynamic Liver MRI
29
Antalya - Turkey
2016
O – 23
Adrenal
Unilateral Adrenalectomy Improves Cushing
Syndrome In a Patient with Primary Bilateral
Macronodular Adrenal Hyperplasia
Alper Çağrı Karcı, Zeynep Çetin, Narin Nasıroğlu İmga, Dilek Berker, Serdar Güler
Department of Endocrinology and Metabolism, Ankara Numune Educational and Research Hospital, Ankara,
Turkey
Primary bilateral macronodular adrenal hyperplasia (BMAH) is a
rare condition, characterized by enlarged adrenal glands and secretion of cortisol independent of pituitary adrenocorticotropic
hormone (ACTH) which results in subclinical or overt Cushing syndrome (CS). Bilateral adrenalectomy is the standard of care in overt
CS but it imposes primary adrenal insufficieny and requires life
long glucocorticoid replacement. We describe a patient with BMAH
who underwent unilateral adrenalectomy of the enlarged adrenal
gland and subsequently displayed remission of CS.
ml showing the etiology of CS as adrenal gland. 24 hour urinary cathecholamines, 17-OH progesterone and DHEA-S levels were normal.
Tests for aberrant receptor expression was normal. With the diagnosis
of CS due to BMAH we decided on unilateral adrenalectomy of the predominantly enlarged gland and the patient underwent laparoscopic
left adrenalectomy. On macroscopic examination adrenal gland size
was 9,5x5x4,5 cm consisting of multipl yellow nodules. Microscopic
examination revealed multiple nodules composed of lipid rich clear
cells and Ki-67 proliferation index was %2.
57 year old woman with a history of type 2 diabetes mellitus (DM)
and hypertension for 5 years, atrial fibrillation and ischemic stroke
for 3 years admitted to our clinic because of bilateral adrenal enlargement on MRI that’s seen due to abdominal pain. On physical
examination, patient was found to have centripedal obesity with
BMI 34 kg/m², plethora and buffalo hump. Her blood pressure was
140/80 mmHg under ramipril 5 mg and carvedilol 12,5 mg treatment. With metformin 2000 mg, insulin aspart 3x10 ü and insulin detemir 1x16 ü the patient’s HbA1C level was %6,7. Contrast
enhanced CT revealed multinodular enlargement of both adrenal
glands with left side predominance and the largest nodules were
40x40 mm on left side and 22x20 mm on right side.
Serum cortisol was 4,3 µg/dl on post operative day 1 and hydrocortisone 25 mg was started. Two months after surgery ACTH stimulation test was normal and hydrocortisone was stopped. One month
later urinary cortisol, late night salivary cortisol were normal and
cortisol was suppressed in response to 2 mg DST and CS was in
remission. At the last visit 8 months after surgery the patient was
in remission, lost 12 kg weight and DM was treated with metformin
alone without insülin requirement and HbA1C level was %5.8. We
follow-up patient with six months intervals.
Endocrine assesment showed 3-4 fold increased urinary cortisol levels, loss of diurnal rhythm with elevated late night serum and salivary
cortisol levels. Plasma cortisol was not suppressed in a 2 mg dexametasone suppression test (DST). Plasma ACTH level was < 5 pg/
Unilateral adrenalectomy of the larger gland appears to be an effective treatment for BMAH. It may result in remission of CS and
improves obesity and diabetes. But follow-up of the patient is required for recurrence of the disease.
Keywords: Cushing syndrome, primary bilateral macronodular
adrenal hyperplasia, unilateral adrenalectomy
Table 1. Laboratuary tests for Cushing syndrome evaluation
AT DIAGNOSIS
POST-OP 3 MONTHS
POST-OP 8 MONTHS
<5 // <5
30
24
17,4
16,2
15,8
24-hour urinary
cortisol (3,5-45 mcg/day)
156 // 210
40
34
Late-night salivary
cortisol(0,2-2,2 ng/ml)
6,8 // 8,83
2,2
1,89
10,8
1,83
1,68
ACTH (pg/ml)
Cortisol (mcg/dl)
2 day-2mg DST
20 - 23 October, 2016
30
Antalya - Turkey
2016
O – 24
Adrenal
CAH Presenting as Premature Puberty with
Associated Testicular Adrenal Rest Tumors
(TART)
Bayar Ahmed Qasim
Dpartment of Medicine, University of Duhok, iraq
Background: Congenital adrenal hyperplasia (CAH) is one of autosomal recessive disorders resulting from CYP 21(21-hydroxylase)
deficiency. Testicular adrenal rest tumors (TARTs) are common in
CAH, due to hyper-secretion of ACTH. These lesions inside the testis are bilateral and multiple. TARTs may lead to structural damage
and their tumors may be mistaken for Leydig cell tumor. Because
of their locations; TART may cause seminiferous obstruction and
infertility.
History: 6-year old boy presented with early growth of pubic hair,
the condition started at age of four years when his parents noticed
that their child has developed pubic hair. They also noticed that he
is taller than his siblings with abnormal behavior and deepening
of voice. There is history of sudden death of his brother when he
was 22 days old. Testicular examination was normal. Physical examination revealed develpoment of pubic hair (stage 4) and adult
scrotum.
20 - 23 October, 2016
Diagnosis and Treatment: His random 17OH progesterone was
60.80 mg/ml (increased by more than 60 folds, repeated result for
17OH progesterone showed increase by more than 20 folds). U/S
and MRI showed testicular tumors keeping with TARTs. We started
patient on HC tabs with consultation of urologist . Continuous follow up of these tumors were done.
Conclusion: Giving the clinical presentation, biochemical profile
and MRI findings, the diagnosis is keeping with CAH complicated
with TARTs. Multidisciplinary approach with the involvement of endocrinologist, pathologist and urologist is of great importance for
the correct diagnosis and treatment. The aim is to draw the attention of the clinicians to the presence of the TARTs and to evaluate
critically every patient with testicular tumors concomitantly with
CAH. It’s very important to consider treatment of CAH to prevent
short stature in future.
Keywords: CAH, TART, precocious pubery
31
Antalya - Turkey
2016
O – 25
Adrenal
Different Clinical Presentations in Three
Patients with Adrenocortical Cancer
Nagihan Beştepe1, Didem Özdemir1, Şefika Burçak Polat1, Mehmet Kılıç2,
Aydan Kılıçarslan3, Melike Ruşen Metin4, Reyhan Ersoy1, Bekir Çakır1
1 Yildirim Beyazit University Faculty of Medicine, Department of Endocrinology and Metabolism Ankara, Turkey
2 Yildirim Beyazit University Faculty of Medicine, Department of General Surgery, Ankara, Turkey
3 Yildirim Beyazit University Faculty of Medicine, Department of Pathology, Ankara, Turkey
4 Atatürk Education and Research Hospital, Department of Radiology, Ankara, Turkey
Introduction: Adrenocortical carcinoma (ACC) is a rare disease
with poor prognosis. About 42% to 57% of patients with ACC present with symptoms of hormonal excess such as Cushing syndrome
or virilization. Non-functional tumors usually present with abdominal or flank pain, varicocele and renal vein thrombosis. In addition,
a growing proportion of these patients (>15%) is initially diagnosed
incidentally. Here we report 3 ACC cases with different clinical presentations.
Case 1: A 38-year-old female patient admitted to our clinic with
low back and abdominal pain, weight loss, and hirsutism. On physical examination, her body mass index was 17.3 kg/m2 and Ferriman-Gallwey score (FGS) was 10. She had a history of vena cava
inferior (VCI) thrombosis and a filter was put in another center 6
months ago. Abdominal ultrasonography (US) revealed invasion
and thrombosis in VCI and multiple metastatic lesions with the biggest diameter of 58 mm in the liver. In computerized tomography
(CT), a 200x95 mm lesion in the left adrenal gland was observed.
Basal cortisol and ACTH levels were 11.4 mcg/dL and 4.6 mcg/dL,
respectively. Overnight 1 mg and 2 days 2 mg dexamethasone suppression tests (DSTs) showed no suppression in the morning fasting cortisols. DHEAS was 907 mcg/dl (normal limits: 0-340 mcg/dl)
(Table 1). The percutaneous biopsy of metastatic mass in the liver
revealed ACC and the patient was evaluated as inoperable.
Case 2: A 57-year-old female patient admitted to internal medicine
clinic with fatigue, abdominal pain and high blood pressure. She
was referred to our clinic due to low potassium (3.1mEq/L) concentration. In further laboratory examination, basal cortisol was 11.8
mcg/dL, ACTH was 2.6 mcg/dL, DHEAS was very high (1000 mcg/
dl) and plasma aldosteron/renin ratio was normal. Abdominal CT
revealed a 110x88 mm mass in the right adrenal gland. There was
no suppression in overnight 1 mg and 2 days 2 mg DSTs (Table 1).
Patient underwent right adrenalectomy and pathology was consistent with ACC.
20 - 23 October, 2016
Case 3: A 52-year-old female patient admitted to our clinic with
hirsutism in the last 6 months. On physical examination, her body
mass index was 31 kg/m2 and plethora, moon face, abdominal obesity, abdominal blue-purple striae, and buffalo hump were present.
Basal cortisol and ACTH were 23.0 mcg/dl and 1.0 pg/ml, respectively. DHEAS was 884 mcg/dl. Overnight single dose of 1 mg and
2 days 2 mg DSTs showed no suppression (Table 1). Adrenal Cushing’s syndrome was diagnosed. A 80x60 mm mass in the left adrenal gland was observed in abdominal CT. Right adrenalectomy was
performed and the histopathological diagnosis was ACC.
Conclusion: ACC is seen more commonly, diagnosed at a younger age and more often functional in females compared to males.
The clinical presentation varies in a wide spectrum ranging from
asymptomatic/mild disease as in case 3 to symptoms related with
hormone overproduction as in case 2 and to severe life threating
metastasis as in case 1.
Keywords: adrenocortical cancer, cushing syndrome, inferior vena
cava trombosis, hypokalemia
Figure 1
Adrenal masses in patients Case 1: 200x95 mm heterogeneous necrotic hypodense solid lesion in the left adrenal gland (CT) Case 2:
110x88 mm heterogeneous necrotic solid lesion in the right adrenal gland (CT) Case 3: 80x60 mm heterogeneous solid lesion in the
left adrenal gland (CT)
32
Antalya - Turkey
2016
Table 1: Clinical, laboratory and radiological findings of patients
Case 1
Case 2
Case 3
Age /sex
38/Female
57/Female
52/Female
Presentation
Abdominal pain, weight
loss, hirsutism, inferior
vena cava thrombosis
Fatigue, abdominal pain
high blood pressure,
hypokalemia
Hirsutism, moon face, bluepurple striae, buffalo hump
Basal cortisol (mcg/dl)
11.4
11.8
23
ACTH (pg/ml)
4.6
2.6
1
Overnight DST (mcg/dl)
10.2
12.4
24
Low dose DST (Liddle) (mcg/dl)
13.1
12.9
25.8
8 mg DST (mcg/dl)
12.6
13.2
23.2
24 hour urinary catecholamines
Normal
Normal
Normal
Aldosterone/renin ratio
Normal
Normal
Normal
DHEAS04 (0-340mcg/dl)
907
1000
884
17-OH progesterone (0.27-1.99) (ng/ml)
6.2
2.5
0.8
Adrenal imaging
200x95 mm heterogeneous
necrotic hypodense solid
lesion in the left adrenal
gland, metastatic lesions
(max 58 mm) in the liver
110x88 mm heterogeneous
necrotic solid lesion in the
right adrenal gland
80x60 mm heterogeneous
solid lesion in the left
adrenal gland
20 - 23 October, 2016
33
Antalya - Turkey
2016
O – 26
Adrenal
Bilateral Cystic Hemorrhagic Adrenal
Incidentalomas
Stavroula A. Paschou1, Konstantinos Tzioras1, Christina Nika1,
Athanasios Papatheodorou2, Vassilis Samaras3, Andromachi Vryonidou1
1 Department of Endocrinology and Diabetes, Hellenic Red Cross Hospital, Athens, Greece
2 Department of Radiology, Hellenic Red Cross Hospital, Athens, Greece
3 Department of Pathology, Hellenic Red Cross Hospital, Athens, Greece
Case Presentation: A 60 years old Caucasian heavy smoker man
presented with cough and backache for four months as well as
gradually worsening weakness, intermittent abdominal pain and
slight hyperpigmentation. Because of the above, the primary care
physician from a Greek island where the patient lived ordered a
CT, which incidentally revealed bilateral lesions in adrenal glands
(8 cm R and 7 cm L) (Fig. 1). He was then referred to our hospital
for further investigation and treatment. On admission, the patient
presented with dehydration, hyperpigmentation, cachexia and orthostatic hypotension. Laboratory evaluation revealed: Glu=115mg/
dl, Creat=1.9mg/dl, K=5.7mEq/l, Na=121mEq/l, F=5 μg/dl,
ACTH=831pg/ml, DHEAS=13mg/dl and Renin=217 pg/ml. Abdominal MRI confirmed bilateral cystic hemorrhagic adrenal lesions,
while a mass of similar imaging characteristics (5 x 4 cm) at the
lower pole of the left kidney was visible. The diagnosis was acute
adrenal insufficiency due to secondary metastases. After hydration
and replacement therapy with hydrocortisone and fludrocortisone
symptoms gradually improved. A brain and neck CT as well as a
bone scan did not reveal any other pathological findings consistent
with malignancy. Biochemical cancer markers were negative too.
A biopsy of the lesion at the lower pole of the left kidney under CT
guidance was performed and resulted in findings consistent with
sarcomatoid renal cell carcinoma. Thus, the patient underwent left
radical nephrectomy-adrenalectomy. The histopathology examination confirmed the diagnosis of sarcomatoid renal cell carcinoma.
Discussion: Adrenal incidentalomas are most commonly unilateral. Nevertheless, bilateral adrenal incidentalomas are reported in
7.8-15% of cases. Most of them represent single discrete bilateral
lesions, while in a small percentage appear as diffuse adrenal hyperplasia or enlargement. The majority of lesions are benign and
non-functional. However, the possibility of congenital adrenal hyperplasia, metastases, hemorrhage, pheochromocytoma, lympho-
20 - 23 October, 2016
ma, infectious or metabolic storage diseases is higher compared
to unilateral lesions. Acute adrenal insufficiency due to adrenal
metastases is uncommon, despite the fact that adrenal glands represent a common metastasis site for several solid tumors. This is
likely due to the sufficient functional compensation by the adrenal
glands and the fact that the signs and symptoms of deficiency are
non-specific and often masked by the symptoms of neoplastic disease. Renal cell carcinomas can metastasize to the adrenal glands
via both the lymphatic and the venous system, but bilateral presentation of adrenal metastases is extremely rare. The optimal strategy for the detection of adrenal metastases including the possibility
of biopsy as well the best treatment option are not clear in the literature and should be individualized. The diagnosis and treatment
approach needs multidisciplinary team collaboration with active
participation of endocrinologists.
Keywords: adrenal, incidentalomas, bilateral, metastases
Figure 1: Chest VT: arrows indicate bilateral adrenal
lesions revealed incidentally
Figure 1. Chest VT: arrows indicate bilateral adrenal lesions revealed incidentally
34
Antalya - Turkey
2016
O – 27
Adrenal
xcAdrenocortical carcinoma presenting
with Cushing’s syndrome in a patient with 8
years history of adrenal insufficiency and
adrenomyolipomas
Tahir Saygın Öğüt1, Gökhan Tazegül1, Hümeyra Bozoğlan2, Özlem Doğan2, Nusret
Yılmaz2, Ramazan Sarı2, Hasan Ali Altunbaş2, İnanç Elif Gürer3,
Gültekin Süleymanlar4, Mustafa Kemal Balcı2
1 Akdeniz University, Faculty of Medicine, Department of Internal Medicine, Antalya/Turkey
2 Akdeniz University School of Medicine, Department of Endocrinology, Antalya/Turkey
3 Akdeniz University School of Medicine, Department of Pathology, Antalya/Turkey
4 Akdeniz University School of Medicine, Department of Nephrology, Antalya/Turkey
Introduction: Primary adrenal cortical carcinoma is a high-grade
tumor associated with poor prognosis. Sixty percent of adrenal carcinomas are functional. Thirty percent of these patients may exhibit
Cushing’s syndrome. This is the first case report in the literature of
an adrenal carcinoma presenting with Cushing’s syndrome developing on 8 year history of adrenomyolipomas and adrenal insufficiency.
Case: A fifty years old male patient was admitted with fatigue and
hyperpigmentation to another instution in 2008, when he was diagnosed with adrenal insufficiency. At the time of diagnosis, abdominal MRI revealed multiple lesions 2 cm mass on left and 5 cm mass
in right adrenal glands consistent with adrenomyolipomas. He was
not investigated further on the etiology of adrenal insufficiency,
and his symptoms were alleviated with prednisolone and fludrocortisone. In February 2016, patient was admitted to another instutions’ Endocrinology outpatient clinic with hypertension, pretibial
oedema, weight gain and weakness in lower extremities. He was
started on antihypertensive medication. Routine laboratory tests
were within normal limits. PET-CT revealed an increase in size of
adrenal tumors; right adrenal mass was increased to 13 cm (SUVmax: 17.75) and left adrenal mass was increased to 5 cm (SUVmax:
14.47). Patient was admitted to our clinic for second opinion. In his
physical examination, his face was plethoric, he had 3+ pretibial
oedema bilaterally. His blood pressure was 160/90 mmHg. Routine
laboratory tests revealed hypokalemia [3.1 mEg/L (3.5-5.1 mEq/L)],
20 - 23 October, 2016
proteinuria (1.700 mg/day) and increased androstenedione levels
[264 ng/dL (40-150 ng/dL)]. Fludrocortisone and prednisolone were
gradually decreased and stopped. Basal cortisol levels were within
normal levels even when the prednisolone was stopped (18.2 µg/
dL). One-mg dexamethasone test did not suppress serum cortisol
(13.3 µg/dL). Midnight cortisol was elevated (12.2 µg/dL). Salivary
cortisol [0.91 µg/dL (0-0.41 µg/dL)] and urinary free cortisol levels
were elevated as well [1207 nmol/day (36-137 nmol/day)]. ACTH
levels were normal [8.76 pg/mL (0-46 pg/mL)]. Patient was diagnosed as Cushing’s syndrome. Patient underwent bilateral adrenalectomy, pathology revealed metastatic adrenocortical carcinoma on right adrenal gland (ki-67: %10, 2 paraaortic lymph nodes
were positive for metastases) and adrenomyolipoma on left adrenal gland. Postoperatively, prednisolone and fludrocortisone doses
were regulated, patient was consulted with oncology; mitotane was
started.
Discussion: There are case reports available in the literature on
adrenomyolipomas causing Cushing’s syndrome; however adrenal
insufficiency has not been previously reported. There are no case
reports of an adrenal carcinoma developing on adrenomyolipomas.
This is a rare and interesting case report of an adrenal carcinoma
presenting with Cushing’s syndrome developing on 8 year history of
adrenomyolipomas and adrenal insufficiency.
Keywords: adrenocortical carcinoma, adrenal insufficiency, adrenomyolipoma
35
Antalya - Turkey
2016
O – 28
Pituitary
Idiopathic Granulomatous Hypophysitis
Mimicking Pituitary Adenoma
Gülay Şimşek Bağır1, Filiz Ekşi Haydardedeoğlu1, Okan Bakıner1, Emre Bozkırlı1,
Özgür Kardes2, Fazilet Kayaselçuk3, Melek Eda Ertörer1
1 Department of Endocrinology, Baskent University, Adana,Turkey
2 Department of Neurological Surgery, Baskent University, Adana, Turkey
3 Department of Pathology, Baskent University, Adana, Turkey
Granulomatous hypophysitis (GH) is a rare disease that mimics
non-functioning pituitary adenomas. At magnetic resonance imaging (MRI), the most common feature is pituitary enlargement.
Histological examination is the gold standard for the diagnosis of
GH. It is characterized by variable numbers of multinucleated giant
cells and lymphocytes, numerous histiocytes, variable amounts of
granuloma formations and fibrosis. Its most common presenting
symptom is headache. In the absence of a demonstrable causative
agent, such as; tuberculosis, sarcoidosis or Langerhans cell histiocytosis, it is termed as idiopathic granulomatous hypophysitis
(IGH). Herein, we present a case with a macroadenoma filling the
pituitary fossa that was diagnosed as IGH following surgery.
A 44-year-old woman was admitted to the hospital with a 5-months
history of headache and oligomenorrhea. Magnetic resonance imaging showed a macroadenoma with suprasellar extension abutting the optic chiasm and the pituitary stalk was deviated to the
right. Her visual field test was normal. Hormonal profile demonstrated the followings: TSH:0.917 uIU/ml (0.4-4.6), freeT4:20.66
pmol/L (11.5-22.7), early morning cortisol:17.75 mcg/dl (4.3-22.4),
FSH:19.11 mIU/mL (1.42-15.4), LH:8.5mIU/mL (1.24-7.8), Estradiol: 19.85 pg/ml (97-216 pg/ml) and IGF-1:149 ng/mL (119-307).
Her prolactin level was normal: 24.6 ng/mL (2.8-29.2). Based on
the laboratory and MRI findings, the initial presumptive diagnosis
of our case was a non-functioning pituitary macroadenoma and
she underwent transnasal-transsphenoidal pituitary surgery. Her
pathologic findings suggested granulomatous hypophysitis. It exhibited granulomatous changes with multinucleated giant cells
and focal areas of necrosis. Ziehl-Nielson (ZN/AFB) and periodic
acid Schiff’s (PAS) stains were done and demonstrated no evidence
of acid fast bacilli or fungal elements. Once the histological find-
20 - 23 October, 2016
ings was definitive, further diagnostic work-up for the exclusion
of systemic granulomatous diseases was performed, including;
polymerase chain reaction (PCR) for Mycobacterium tuberculosis
in tissue sample, purified protein derivated (PPD) test and computed tomography (CT) of the thorax. Measurement of serum angiotensin converting enzyme (ACE) and ophthalmologic examination
were done for ruling out sarcoidosis. All the tests were found to be
negative. Additionally, any autoimmune background was found. Depending on the findings, the final diagnosis of our patient was IGH.
Idiopathic granulomatous hypophysitis is a rare inflammatory disease of unknown etiology. It‘s not clear whether lymphocytic hypophysitis and IGH are separate diseases or are distinct presentations of the same disease. There is also some evidence in the
literature that IGH is an autoimmune pituitary disorder. It is difficult to diagnose IGH preoperatively and is often misdiagnosed. Although rare, it should be kept in mind in the differential diagnosis
of sellar lesions.
Keywords: Hypophysitis, Granulomatous, adenoma
Figure 1: Histological photomicrographs
Granuloma with multinucleated giant cell (arrow), H&E x200 (Figure a), H&E x400 (Figure b) Histiocytes showing positive CD68 with
immunohistochemical staining x 200 (Figure c)
36
Antalya - Turkey
2016
O – 29
Pituitary
Case of giant prolactinoma in young man
Alexandr Tsiberkin, Tatiana Karonova, Anna Dalmatova, Natalia Klyaus,
Elena Grineva
Institute of Endocrinology, Federal Almazov North-West Medical Research Centre, Saint Petersburg, Russia
This case illustrates progress of a giant prolactin-secreting pituitary adenoma in young man with a family history of prolactinomas
in the absence of the adequate therapy for 8 years after initial diagnosis.
secondary adrenal insufficiency, hypogonadotropic hypogonadism
and secondary hypothyroidism. Cabergolin therapy with a gradual
increase of dose up to 1.0 mg per week was initiated as well as
replacement therapy with L-thyroxine.
The 36 year-old Caucasian man admitted with complaints of intense headache and progressive visual deterioration. These complaints started at age 26 and MRI revealed pituitary macroadenoma
at a size of 44*44*29 mm with severe extrasellar extension with
chiasmal compression and involving right cavernous sinus. His serum prolactin level was 202.8 μg/L (4.9-25.4). The patient refused
offered treatment via transcranial surgery with subsequent radiotherapy. Further, the patient did not seek any medical treatment for
8 years. Increase the intensity of the headache prompted patient to
repeat MRI which revealed continued tumor grow to 65*52*70 mm
with dislocation of brain median structures with 5 mm.
After 2 months serum prolactin decreased to 10849.0 μg/L (4.015.2) and cabergoline dose was increased to 2.0 mg per week. Six
month after initiation of treatment with cabergoline patient noted a
regression of headache and improvement of visual function, serum
prolactin level decreased by 36 times and reached 469.4 μg/L (4.015.2), tumor shrinkage reached 25%.
Goldmann perimetry showed bitemporal hemianopsia. According to laboratory analysis serum prolactin level in probe with dilution was 17090.0 μg/L (4.0-15.2). Also there was sign of latent
20 - 23 October, 2016
Described case emphasize the crucial role of identification of hyperprolactinemia among young men on early stages of the disease
and necessity of the proper treatment by dopamine agonists after
verification of diagnosis. Cabergoline therapy is effective even in
case of giant prolactinomas.
Keywords: giant prolactinoma, invasive tumor, panhypopituitarism, cabergoline.
37
Antalya - Turkey
2016
O – 30
Pituitary
Pituitary Stalk Interruption Syndrome: A
Rare Cause of Panhypopituitarism
Filiz Ekşi Haydardedeoğlu, Gülay Şimşek Bağır, Okan Sefa Bakıner, Emre Bozkırlı,
Melek Eda Ertörer
Department of Endocrinology, Baskent Üniversity, Adana,Turkey
Case: A 36 year old woman was admitted to our hospital with the
complaint of fatigue. She had the story of medical follow-up at another hospital for 10 years because of pituitary insufficiency. She
was taking hormonal replacement therapy for hypocorticolism,
hypothyroidism and primary amenorrhea since she was a child.
She experienced pubertal changes and had regular menstruation
under cyclic estrogen/progesteron replacement. Detailed history revealed that the patient was born by vaginal delivery after full
term pregnancy period. At birth, her weight and height were normal, 3600 grams and 52 cm, respectively. There was no history of
consanguinity between her parents.
She had no spesific complaint on admittance. Height was 157 cm,
and weight was 54.4 kg. Her breasts were at Tanner 3 stage, axillar
and pubic hair were scarce. Her laboratory examination revealed
low IGF-1; 26.7 ng/ml (109-284), elevated prolactin; 54.86 ng/ml
(2.8-29.2), suppressed TSH 0.01 UIU/ml with normal FT4;17.89
pmol/L (11.5-22.7) and low morning cortisol; 0.5 µg/dl (4.30-22.40).
Since the patient was using estrogen/progesteron, gonadal axis
was not evaluated. On pituitary MRI, the stalk was not observed
before and after contrast administration and neurohypophysis was
found to be located ectopically. According to clinical and radiological results, the patient was diagnosed as pituitary stalk interruption syndrome.
Figure 1. Pituitary images in coronal plane
Because of the high conccurance rate of malformations especially
in the central nervous system and craniofacial structures, cranial
MRI was performed and cerebellar tonsiller ectopia was reported
besides ectopic neurohypophysis and absence of pituitary stalk.
Figure 2: Pituitary images in saggital plane
Discussion: Pituitary stalk interruption syndrome is a rare congenital defect and the cause has not been fully understood. The
diagnosis depends on MRI findings. This syndrome causes varying degrees of anterior pituitary hormone deficiencies and related complaints. Hypoglycemia during neonatal period, growth retardation and lack of pubertal development with age are the main
contributors of clinical presentation. Lifetime hormonal replacement therapy is required in these patients. The MRI findings of pi-
20 - 23 October, 2016
38
Antalya - Turkey
2016
tuitary stalk interruption syndrome should fully indicate pituitary
hormonal evaluation. Although the presence of malformation does
not seem to be associated with severity of hormonal deficiencies,
20 - 23 October, 2016
these patients should be evaluated for the extrahypophyseal malformations, as well.
Keywords: Pituitary, stalk, interruption, panhypopituitarism
39
Antalya - Turkey
2016
O – 31
Neuroendocrine Tumors
How multifocal insulinomas can be diagnosed
and treated in a case with MEN-1 disease?
Sayid Zuhur1, İdris Kuzu2, Yüksel Altuntaş2
1 Department of Endocrinology and Metabolism, Namik Kemal University faculty of medicine, Tekirdag, TURKEY
2 Department of Endocrinology and Metabolism, Sisli Etfal Training and Research Hospital, Istanbul, turkey
Background: Insulinomas can be multifocal and each insulinoma
may arise at different times in cases with MEN-1. Therefore, localization studies and treatment may fail.
Case Presentation: The patient was a 27 years old female, admitted for dizziness, occasional palpitation, feel of hunger, sweating,
and headache, occurring during fasting. Laboratory findings revealed a fasting plasma glucose (FPG) of 47 mg/dL, insulin of 17.9
mIU/mL, C-peptide of 2.85 ng/mL, Ca of 12.1 mg/dL, P of 2.24 mg/
dL, PTH of 272 pg/mL and 25 OHD3 of 4 ng/m L. Plasma glucose,
serum insulin and C-peptide levels were 36 mg/dL, 15.4 mIU/mL
and 2.82 ng/mL, at the time of symptomatic hypoglycemia during
prolonged fasting test, respectively. A pituitary adenoma was found
on MRI. A diagnosis of MEN-1 disease was made. She was commenced on therapy with diazoxide 100 mg bd.
Imaging Studies: Abdominal ultrasonography (USG) and MRI were
normal. Endoscopic ultrasonography (EUS) revealed a 9 mm hypoecoic area in the tail of pancreas (Figure 1). The result of fine
needle aspiration biopsy of the lesion was consistent with pNET
(Figure 4). Parathyroid Tc-99m pertechnetate sestamibi/SPECT CT
revealed radiotracer uptake at the level of C7 vertebrae.
Treatment, Outcome, and Follow-Up:On July 2010 left upper,
lower and right upper glands parathyroidectomy with transcervical thymectomy was performed. However, the right lower parathyroid gland could not be found. Histopathological analysis revealed
a parathyroid adenoma inside the thymus. A distal pancreatectomy was performed on October 2010. Histopathological evaluation
demonstrated 3 distinct well differentiated pNETs (WHO grade I).
The largest tumor was 8 mm in diameter and was immunostained
positively for glucagon and the other two tumors were 6 and 5 mm
in diameters and both were immunostained positively for insulin
(Figure 5,6). On postoperative day 1, the serum Ca, P and PTH levels were 10.6 mg/dL, 2.7 mg/dL and 37.4 pg/mL, respectively. FPG
levels was > 80 mg/dL immediatly after surgery.
She was commenced again on therapy with diazoxide 100 mg bd,
pending imaging results. However, the results of abdominal USG,
MRI, EUS and octreoscan, were unremarkable. The follow-up biochemical analysis results is presented in Table 1.The patient refused surgery.
On January 2016 she developed symptomatic hypoglycemia because she stopped diazoxide treatment. Imaging studies including EUS could not show a pNET. 68Ga DOTATATE PET/CT (Figure
7) showed three focal radio tracer uptakes in the head, body and
tail of the pancreas. She developed severe pretibial edema enough
to cause difficulty in walking. Therefore, the dose of diazoxide was
reduced to 100 mg/day. Now, what kind of surgery should be preferred in this case? enucleation of the tumors or a pancreatectomy?
Keywords: Multifocal insulinomas, Glucagonoma, 68Ga DOTATE/
PET CT, endoscopic ultrasonography
Figure 1
Figure 1. Tc99m SPECT CT showing a parathyroid adenoma located
in the thymus.
On October 2011, she was admitted again for neuroglycopenic
symptoms. Serum insulin and C-peptide levels were 8 mIU/mL and
1.54 ng/mL while FPG was 38 mg/dL on supervised fasting test.
20 - 23 October, 2016
40
Antalya - Turkey
2016
Figure 5. Immunocytochemistry showing two insulinomas.
Figure 2. Head MRI Showing a pituitary adenoma of 4x4 mm
Figure 6. Immunocytochemistry showing a glucagonoma
Figure 3. Endoscopic Ultrasonography showing a 9 mm hypoechoic
tumor in the tale of pancreas
Figure 7. 68Ga DOTATATE PET/CT showing a tumor in the head of
pancreas
Figure 4.Fine needle aspiration biopsy showing dens neuroendocrine cells
20 - 23 October, 2016
41
Antalya - Turkey
2016
Figure 8. Ga68 DOTATATE PET/CT showing two tumors in the body and tale of the pancreas
Table 1
2010
2010
(Post-op)
2011
2012
2014
2015
2016
47
80
72
76
66
71
42
FPG (mg/dL)
Insulin (µIU/mL)
17.9
6.92
15.4
15.1
20
12
C-peptide (ng/mL)
2.85
1.86
2.33
2.74
3.51
2.54
iPTH (pg/mL) (<65)
272
37.4
14.2
120
62
66
70.8
Ca (mg/dL)
12
10.06
11.19
11.5
10.08
9.79
9.94
P (mg/dL)
2.24
2.79
1.83
2.3
2.9
2.9
3.3
4
25
24.17
27
27.2
25OHD3 (ng/mL)
Gastrin (pg/mL) (<115)
Urinary 5-HIAA (mg/24h)
50.02
39.8
4.7
Lon term follow-up laboratory results of the patient
20 - 23 October, 2016
42
Antalya - Turkey
2016
O – 32
Neuroendocrine Tumors
Catastrophic Cushing’s Syndrome:
Report of a mortal case
Seda Oğuz1, Cenk Yücel Bilen2, Cenk Sökmensüer3, Tomris Erbaş1, Selçuk Dağdelen1
1 Department of Endocrinology and Metabolism, Hacettepe University Medical School, Ankara, Turkey
2 Department of Urology, Hacettepe University Medical School, Ankara, Turkey
3 Department of Pathology, Hacettepe University Medical School, Ankara, Turkey
Introduction: Thymic neuroendocrine tumors are rare and they
can be complicated by endocrine abnormalities. Although overall
survival rates are poor, cure is possible with complete surgical resection.
Case Presentation: 30 year old male was admitted to hospital with
sudden onset chest pain, shortness of breath and fever. Diagnosis
of pericardial effusion was made and the patient was transferred to
our center’s cardiovascular surgery department for further evaluation. Laboratory workup was unremarkable except mild leukocytosis and elevated transaminases. Haemorragic effusion was drained
by pericardiosynthesis. No malignant cells were seen on cytologic
exam, tuberculosis culture was negative, as well as viral and reumatologic markers. To visualize thoracic cavity and adjacent structures, cardiac MRI was performed and 38x25x37 cm mass located
to anterior mediastinum which was suggestive of thymic hyperplasia was detected. Surgical treatment recommendation was refused
by the patient. One year later, the mass was enlarged to 45x35x65
mm, surgery was suggested again but the patient did not accept
the operation since he had no complaints.
At the age of 35, he was admitted to general surgery clinic for
swelling and compression feeling on his neck. Large thyroid nodules were seen at US and he was diagnosed as PTC by total thyroidectomy. Several days after discharge, the patient came back
with chest pain, dyspnea, ortopnea and edema. CT determined
110x54x38 mm thymic mass which was invasive to vena cava superior, possible metastatic nodules in the lung, mediastinal and
20 - 23 October, 2016
abdominal enlarged lymph nodes. He was hospitalized for worsening symptoms plus emerging severe muscle weakness. Laboratory
work-up revealed deep hypokalemia, mild leukocytosis and new
onset diabetes. Due to his medical history of PTC, endocrinology
consultation was done. The patient was hypertensive, ortopneic,
had dark skin color with acne formations. These physical and laboratory findings were suggestive of ectopic Cushing’s syndrome. He
had no family history of MEN syndromes. Serum ACTH and cortisol
levels were 450 pg/mL and 102 µg/dL, respectively, while 24 hour
UFC level was 4286 mcg/d. Biopsy of the thymic mass resulted
as high grade neuroendocrine carcinoma. During hospitalization,
thrombosis occured in the right cephalic vein and antibiotic therapy
was started for hospital acquired pulmonary infection. Depending
on the catastrophic Cushing’s syndrome’s life-threatening results,
he was given to bilateral adrenalectomy. Twelve days after surgery,
the patient died as a result of pneumonia.
Discussion: Although neuroendocrine ACTH-producing tumors of
the thymus usually present with signs and symptoms of Cushing’s
syndrome, our patient was known to have thymic mass for five
years, but had ectopic Cushing’s syndrome only at late term, when
the tumor was metastatic. Bilateral adrenalectomy was performed
to save the patient from catastrophic Cushing’s, although he died
of severe infection.
Keywords: Ectopic Cushing’s syndrome, catastrophic Cushing’s
syndrome, thymic neuroendocrine neoplasia.
43
Antalya - Turkey
2016
O – 33
Diabetes
Effect of Imatinib on Insulin Therapy in a
Patient with Type 2 DM
Bengür Taşkıran1, Güven Barış Cansu1, Mustafa Karagülle2, İnci Vetem Karagülle3
1 Deparment of Endcorinology, Yunus Emre State Hospital, Eskisehir, Turkey
2 Deparment of Haematology, Yunus Emre State Hospital, Eskisehir, Turkey
3 Deparment of Internal Medicine, Yunus Emre State Hospital, Eskisehir, Turkey
A 36 year old female patient visited endocrinology outpatient clinic for
type 2 DM. She had DM for 10 years, hypertension, rheumatoid arthritis,
and thyroidectomy for multinodular goiter. She was on insulin therapy
for 7 years. Her mother also had type 2 DM. She denied chronic steroid
usage. She did not have any pancreatitis attack. She was on 50 U/day
glargine insulin, 10 U tid aspart insulin, perindopril 5 mg qd, amlodipin
5 mg qd, levothyroxine 112.5 mcg qd therapy. She was not using metformin due to adverse effects. She weighed 70 kg and had height of 149
cm. Physical examination was normal despite obesity. Acanthosis was
absent. Then she developed CML (chronic myeloid leukemia). First, she
was treated with hydroxyurea 500 mg tid and then medical therapy for
CML was switched to imatinib. Although she had been on high dose insulin therapy, insulin requirement decreased dramatically to 16 U/day
glargine insulin soon after imatinib therapy. Pioglitazone was added to
the therapy in order to counteract insulin resistance. Two months after
pioglitazone therapy glargin dose decreased to 12 U/d.
Causes of decreased insulin requirement are liver/renal failure,
weight loss, decreased appetite, cortisol deficiency, growth hormone deficiency, hypothyroidism, and drugs (non-selective beta
blockers, ACEI). Tyrosine kinase inhibitors (imatinib, dasatinib, and
sunitinib) are indicated for treatment of metastatic thyroid cancer,
CML, and gastrointestinal stromal tumours. Reduced insulin resistance and increased β-cell mass are the proposed mechanisms for
improved glycaemia.
Since she did not have symptoms of primary/secondary cortisol deficiency (malaise, weakness, decreased appetite, hyperpigmentation,
nausea-vomiting, diarrhea), cortisol and ACTH tests were not ordered.
She did not have head trauma suggesting hypopituitarism. Since growth
hormone deficiency leads to hypoglycaemia more frequently in children
compared to adults without other accompanying hypophyseal hormone
deficiencies, IGF-1 was not studied. Hypoglycaemia incidents decreased
with decrements in daily insulin dose. Therefore we did not suspect an
insulinoma. Her weight was almost stable and liver and renal function
tests were normal. Her appetite was good. Near-normal TSH level excluded hypothyroidism as a possible cause for hypoglycaemia and decreased insulin demand. Perindopril may enhance hypoglycaemic effects of anti-diabetic drugs and lead to hypoglycaemia in less than 1% of
treated patients. Our patient was already on perindopril therapy, so we
did not assume it as a contributing factor. We did not evaluate C-peptide
and insulin levels regarding the fact that her ongoing insulin therapy
would affect assessment of the results.
Cancers are usually known as “hyperglycaemic disease states” Hypoglycaemia in a cancerous patient even in case of accompanying
DM, should not be overlooked and simply assumed to result from
poor intake and decreased demand for anti-diabetic medication
Keywords: imatinib, diabetes mellitus, tyrosine kinase inhibitors,
hypoglycemia
Table 1
03/2012
10/2015
glucose (mg/dl)
217
353
creatinine (mg/dl)
0.68
0.75
ALT (U/l)
16
18
HgbA1c (%)
7.17
WBC (/mm3)
10440
40550
Neu (mm3)
7930
34070
Hgb (g/dl)
11.1
13.1
Hct (%)
32.8
38.6
TSH (mIU/l)
5.49
vit B12 (pg/ml)
383
ferritin (ng/ml)
48.1
ESR (mm/hr)
95
22
total protein (g/dl)
7.7
6.7
albumin (g/dl)
4.3
3.6
HbsAg
negative
AntiHCV
negative
laboratory data before and during imatinib therapy
11/2015
180
0.58
19
12/2015
127
0.70
10
01/2016
173
0.69
10
13050
10410
12.2
36.2
5330
3270
11.2
33.7
6650
4270
11.6
35.0
56
6.9
4.0
85
6.6
3.8
49
6.8
4.2
20 - 23 October, 2016
44
Antalya - Turkey
02/2016
196
0.72
10
7.4
5640
3390
12.3
36.6
5.48
38
7.0
4.3
06/2016
138
0.65
11
5360
2870
11.8
34.2
4.31
153
62.7
44
6.92
4.3
negative
negative
2016
O – 35
Adrenal
A Case Of Von Hippel-Lindau Disease
With Bilateral Pheochromocytoma,
Abdominal Paraganglioma And Pancreatic
Neuroendocrine Tumor
Sinem Kıyıcı1, Burçin Uygun1, Semra Aytürk2
1 Department of Internal Medicine, Saglik Bilimleri University, Yüksek İhtisas Education and Research
Hospital, Bursa, Turkey
2 Department of Endocrinology and Metabolism, Trakya University Medical Faculty, Edirne, Turkey
Introduction: Von Hippel-Lindau (VHL) disease is an autosomal
dominant inherited multisystem familial tumor syndrome predisposing to various benign or malignant tumors such as central nervous system and retinal hemangioblastomas, renal cell carcinoma
and cysts, pancreatic tumors and cysts, pheochromocytomas, and
endolymphatic sac tumors.
Case: 40-year-old woman was referred to our clinic with bilateral
adrenal masses diagnosed at abdominal CT which was performed
for lumbar pain. Her medical history was normal. There was no
history of hypertension or hypertensive attack. But her brother had
a history of bilateral adrenalectomy at 30-years-old. There wasn’t
any abnormal finding in her physical examination. Her blood pressure was 120/80 mm/Hg and pulse rate was 72 beats per minute
with a regular rhythm. In the abdomen CT there was a 4 cm nodular
lesion on the left adrenal gland and a 2.5 cm nodular lesion on the
right adrenal gland. Also there was a 2.5 cm nodular lesion around
celiac trunk and a 3 cm nodular lesion at the neighborhood of pancreas head. 24-hour urinary fractionated normetanephrine level
was about 20 times higher than the normal range (8577 µg/24h,
normal value: 88-444 µg/24h) and 24-hour urinary metanephrine
level was 157 µg/24h (normal value: 852-341 µg/24h). Plasma
normetanephrine level was also found increased (14.88 nmol/L,
normal value: 0.12-1.18) while metanephrine level is normal. After the diagnosis of pheochromocytoma, she was operated following appropriate medical preparation. Pathological examination
20 - 23 October, 2016
of the bilateral adrenalectomy material was pheochromocytoma,
paraaortic mass excision was paraganglioma and the pancreatic
nodular lesion was neuroendocrine tumor. She was screened clinically for multiple endocrine neoplasia (MEN) type 2A and there was
no evidence for primary hyperparathyroidism and medullary thyroid carcinoma. We evaluated our patient for VHL disease because
of the young age at presentation, presence of family history for the
pheochromocytoma, coexistence of bilateral pheochromocytoma,
abdominal paraganglioma and pancreatic neuroendocrine tumor.
Central nervous system hemangioblastomas, renal cell carcinomas or renal cysts were not detected. Genetic screening was also
made. Gene mutation for the VHL disease found positive.
Conclusion: Pheochromocytomas are seen both sporadically and
in association with a number of genetic syndromes, including
VHL disease, MEN 2, neurofibromatosis type 1, and mutations of
the succinatede hydrogenase subunits B, D, and C. VHL disease
is a rare hereditary syndrome characterized by benign or malignant tumors. Pheochromocytomas in VHL disease tend to be seen
in younger patients, are often bilateral, may be extraadrenal, and
are less likely to be associated with symptoms of catecholamine
production compared with those occurring in patients without VHL.
Genetic screening should be considered for the patients with pheochromocytomas to proper treatment and the follow-up.
Keywords: pheochromocytoma, adrenal, genetic
45
Antalya - Turkey
2016
0 – 36
Adrenal
Silent Pheochromocytoma in von HippelLindau Disease: Report of a Case
Rıza Can Kardaş1, Seda Oğuz2, Ahmet Bülent Doğrul3, Cenk Sökmensüer4,
Selçuk Dağdelen2
1 Hacettepe University School of Medicine, Department of Internal Medicine, ANKARA, TURKEY
2 Hacettepe University School of Medicine, Department of Internal Medicine, Division of Endocrinology and
Metabolism , ANKARA, TURKEY
3 Hacettepe University School of Medicine, Department of General Surgery , ANKARA, TURKEY
4 Hacettepe University School of Medicine, Department of Pathology , ANKARA, TURKEY
Introduction: Von Hippel-Lindau (VHL) disease is characterized
by (1) hemangioblastomas involving the retina, cerebellum, brain
stem, and spinal cord; and (2) visceral malignancies, such as renal
cell carcinomas, pheochromocytomas, and pancreatic neuroendocrine tumors. Here we present a case of VHL disease, who initially
presented with mixed-type deafness and hypothyroidism and then
diagnosed with VHL disease via familial screening.
Case Presentation: A 19-year-old woman was diagnosed with
VHL-disease during familial screening, after her mother, the index case, was also diagnosed with VHL-disease. Her mother had
initially presented to the clinic with numbness in her left arm and
during the course of her disease, she was diagnosed with cerebellar hemangioma, renal cell carcinoma, bilateral pheochromocytoma, and non-functional neuroendocrine tumor of the pancreas.
Both she and her daughter were found to have heterozygous c.353
T>G mutation in the VHL gene.
Before her genetic diagnosis, she had first presented with delayed
speech and was diagnosed with bilateral mixed type hearing loss
when she was 3 years old. Later in the course of her routine outpatient follow-up, she was diagnosed with Hashimoto’s thyroiditis
when she was 12 years old and levothyroxine replacement was
started. After the patient was found to be heterozygous for the mutation, potential neoplasms were checked. She was normotensive
and asymptomatic except for her hearing loss, and both physical
and ophthalmological examinations were unremarkable. Cranial,
spinal, and cochlear MRI revealed no abnormalities. After bilater-
20 - 23 October, 2016
al hypoechoic adrenal masses were found on the initial abdominal
ultrasonography, abdominal MRI was performed and it confirmed a
17×15 mm right and a 29×26 mm left adrenal mass, both of which
were hyperintense in T2A sequences and showed notable contrast
uptake. Both the pancreas and the kidneys were normal.
Further studies to delineate the nature of the adrenal masses were
performed. 24-hour urine metanephrine, normetanephrine, and
vanilmandelic acid levels were all in normal ranges and 68Ga-DOTATATE PET imaging showed increased uptake at both adrenals.
Based on the functional imaging results at VHL background, bilateral (silent) pheochromocytoma was diagnosed and bilateral
adrenalectomy was performed. Immunohistochemistry showed
negative staining for S100 and pan-CK staining and positive for synaptophysin and chromogranin, all of which were consistent with the
diagnosis of pheochromocytoma.
Discussion: Genetic testing plays an important role for the workup of the VHL disease. Here we presented an asymptomatic case
of VHL-disease, diagnosed via genetic testing, and was found to
have bilateral pheochromocytomas. Hearing loss can be seen with
endolymphatic sac tumors associated with VHL disease, but cranial and cochlear imaging revealed no abnormalities in this patient.
A review of literature showed no reports of association between
VHL-disease and hypothyroidism.
Keywords: von Hippel-Lindau disease, pheochromocytoma, hypothyroidism, hearing loss
46
Antalya - Turkey
2016
0 – 37
Adrenal
Adrenocortical carcinoma in pregnancy
Seher Tanrıkulu1, Sakin Tekin1, Ayşe Kubat Üzüm1, Nihat Aksakal2,
Tuğba Saraç Sivrikoz3, Semen Önder4, Ferihan Aral1
1 Department of Endocrinology, Istanbul University, Istanbul, Turkey
2 Department of General Surgery, Istanbul University, Istanbul, Turkey
3 Department of Gynecology and obstetrics, Istanbul University, Istanbul, Turkey
4 Department of Pathology, Istanbul University, Istanbul, Turkey
Adrenocortical carcinoma(ACC) is very rare tumors and much more
uncommon with pregnancy. Only few case reports are reported in
the literature because of the decreased fertility in patients with
ACC in addition to rarity of the disease. We present the case of a
31- year-old pregnant woman with ACC.
Case: A 31-year-old woman in her second pregnancy was admitted
to the hospital with sudden onset flank pain in her left upper quadrant at 33 weeks’ gestation.
She was normotensive (125/75mmHg) and physical examination
was normal.
Laboratory studies revealed Hb: 9,2 g%, Hct: 28,5%, PLT: 161000 /
mm3, Glu: 129 mg/dL (random), Cr: 0.4 mg/dL, K:3.7 mmol/L, Na:
139 mmol/L, albumin: 3,2 gm/dL (3.5-5.5), LDH: 637 U/L (135-250).
Abdominal ultrasonography and MRI revealed a large heterogeneous tumor in the left adrenal gland measuring 145x100 mm.
Fetal development was normal as per gestational age. A detailed
endocrine assessment was done. At the first week of hospitalization preterm labor began and the patient underwent cesarean sec-
20 - 23 October, 2016
tion. The female baby was healthy, weighing 2350 g, Apgar score
9/10. The patient was normtensive and normoglycemic during her
stay in the hospital. 24-hour urine for metanephrines and normetanephrines were normal. Morning cortisol level measured was 21,6
mcg/dl (4-19,5), ACTH: 7,6 and 13,8 pg/mL. Low-dose dexamethasone suppression test revealed cortisol level 7,9 mcg/dL. Testosterone and DHEA-S levels were 0,608 ng/mL (0,05-0,52) and 202
μg/dL (35-430), respectively. 17-OH Progesterone was 15.8 ng/ml
and TSH was 3,13 μIU/mL (0,2-4,2). The patient was evaluated by
a multidisciplinary team and underwent to laparoscopic surgery.
Pathological evaluation revealed, left adrenocortical carcinoma
with a Ki score of 30%.
ACC can cause maternal and fetus mortality. Tumor size, Ki-67 index, stage, and resection status are important prognostic indicators of survival in ACC patients. Prognosis of ACC during pregnancy
is reported to be poorer than nonpregnant women. Hence early disease recognition is essential.
Keywords: adrenocortical carcinoma, pregnancy, laparoscopy
47
Antalya - Turkey
2016
0 – 38
Adrenal
A case with adrenal cyst hydatid mimicking
malignant tumour in MRI and PET-CT
Hüsniye Başer1, Oya Topaloğlu2, Neslihan Çuhacı2, Mehmet Kılıç3, Niüufer Poyraz4,
Hilal Ahsen5, Meltem Yıldırım6, Reyhan Ersoy2, Bekir Çakır2
1 Department of Endocrinology and Metabolism, Ataturk Education and Research Hospital, Ankara, Turkey
2 Department of Endocrinology and Metabolism, Yildirim Beyazit University Faculty of Medicine, Ankara, Turkey
3 Department of General Surgery, Yildirim Beyazit University Faculty of Medicine, Ankara, Turkey
4 Department of Nuclear Medicine, Ataturk Education and Research Hospital, Ankara, Turkey
5 Department of Pathology, Ataturk Education and Research Hospital, Ankara, Turkey
6 Department of Radiology, Yildirim Beyazit University Faculty of Medicine, Ankara, Turkey
Introduction: The adrenal gland is considered as an exceptional
localization of a hydatid cyst. Adrenal hydatid cysts are usually secondary to generalized echinococcosis. The reported incidence of
adrenal hydatid cysts is 0.5%. We present here a case of an adrenal
hydatid cyst mimicking a malignant tumour in the imaging techniques such as magnetic resonance imaging (MRI) and positron
emission tomography (PET)- computerized tomography (CT).
Case: A 70-year-old woman was admitted to our hospital for abdominal pain. She had undergone surgery for liver hydatid cysts
4 years ago. No hypertension was present in medical history. In
her physical examination; blood pressure, temperature, and heart
rate were 130/90 mmHg, 36.9ºC, and 88/min, respectively. Tenderness in the epigastrium and right hypochondrium were observed
on palpation. Other physical examination findings were normal.
Blood tests revealed leukocytes and eosinophils within normal
values except anemia (Hb:11.2 gr/dL). Erythrocyte sedimentation
rate was 93 mm/h. Abdominal ultrasonography revealed 71x91
mm and 61x65 mm hypoechoic masses containing internal cystic
components in the liver. Abdominal MRI showed that there were
cystic masses with 8 cm and 9 cm in diameter in the right lobe of
the liver, and a 65x40 mm heterogenous probably malignant mass
with internal cystic components in the right adrenal localisation
which was showing diffusion limitation and contrast enhancement
in early and delayed phases (Figure 1). Indirect hemaglutination
test of echinococcus granulosus was positive. Renin, aldosterone,
plasma metanephrine and normetanephrine, fractionated urinary
catecholamine and dehydroepiandrosterone sulfate levels were
normal. One mg dexamethasone suppression test was evaluated
as negative. In PET-CT, increased 2-[fluorine-18]-fluoro-2-deoxy-D-glucose (FDG) involvement was observed in the right adrenal mass (SUVmax 8.3), and cystic lesions of liver (SUVmax 4.8).
She underwent an open laparotomy, and drainage of hepatic cysts
20 - 23 October, 2016
and right adrenalectomy were performed. Histopathological examination revealed a cuticular layer and germinative membrane.
Conclusion: Hydatid cystic disease of the adrenal glands is rarely
observed in clinical practice. Most hydatid cysts arising from the
adrenal gland are part of a disseminated disease. These are mostly asymptomatic and found incidentally by imaging or during surgery for other abdominal pathologies. Treatment of adrenal hydatid
cysts is mostly surgical (pericystectomy or resection of the entire
adrenal gland). The adrenal cyst hydatids can mimic malignant adrenal lesions in imaging techniques as in our case. Adrenal hydatid
cysts should be considered in patients with incidentally discovered
adrenal masses especially with a history of hydatid cystic disease.
Keywords: adrenal gland, liver, hydatid cystic disease, imaging
techniques
Figure 1. Post-contrast T1-weighted axial MRI shows lesion containing heterogenous contrast accumulation
48
Antalya - Turkey
2016
O – 39
Pituitary
Hamartoma of Hypothalmus Presented As
Precocious Puberty and Epilepsy in 10-years
Old Girl
Bayar Ahmed Qasim
department of medicine, university of Duhok, IRAQ
Background: Hamartoma of the hypothalamus represents a wellknown but rare cause of central precocious puberty and gelastic
epilepsy. Due to the delicate site in which the tumor is located, surgery is often difficult and associated with considerable risks.
History: 10-year old girl presented with early menstrual cycles.
The condition started at age of one year when her parents noticed
that their child has developed abnormal vaginal bleeding. Her cycles were regular, each cycle lasted for 3 days. Her parents also
noticed that she has developed breast, axillary and pubic hair at the
age of five, and seven respectively. The parents also gave history of
difficulty in speech especially articulation and abnormal generalized body movements (epilepsy) since childhood for which she is on
medications. She also had attacks of inappropriate laugh.
20 - 23 October, 2016
Diagnosis and Treatment: Her lab tests consisted with central
precocious puberty, MRI shows suprasellar mass and she had been
put on leuprolide and antiepileptic medicines until surgery planned.
Her surgery was done with complete resection, with histopathology
showing hamartoma. After surgery there was complete remission
of seizure.
Conclusion: The treatment of hypothalamic hamartoma (HH) associated with generalized epilepsy (GE) has been found to improve
seizures and behavioral disturbances with an acceptable morbidity
rate by using a variety of surgical approaches. Partial resection of
tumor may be sufficient to reduce seizure frequency and to improve
behavior and quality of life with few side effects.
Keywords: hypothamus, precocious puberty, hamartoma
49
Antalya - Turkey
2016
O – 40
Pituitary
A rare cause of Diabetes Insipidus: Rosai
Dorfman Disease
Adile Begüm Bahçecioğlu1, Alper Gürlek2
1 Hacettepe University School of Medicine, Department of Internal Medicine , ANKARA, TURKEY
2 Hacettepe University School of Medicine, Department of Internal Medicine, Division of Endocrinology and
Metabolism , ANKARA, TURKEY
Introduction: Rosai Dorfman disease (RDD) is a benign non-Langerhans cell histiocytosis with multiorgan involvement. The connection between histiocytosis and central diabetes insipidus (CDI) was
first reported in the late 1800s, and it has been a rare cause of CDI
since.
Case Presentation: 43-year old female was refered to our endocrinology department because of polyuria and polydipsia. Water restriction test was consistent with central diabetes insipidus. Laboratory tests showed no abnormality of anterior pituitary hormones.
Desmopressin treatment was initiated. One year later she applied
because of diplopia, fatigue, fever, night sweats and skin lesions.
Skin lesions first started as periorbital pruritus and dryness, than
expanded to perioral region, cheek, dorsum and chest in several months. Physical examination revealed yellowish papules and
plaques on periorbital region an bilateral cervical lymphadenopathy. Punch biopsy of the skin lesions resulted as polymorphous
infiltrate of lymphoid cells which were predominantly histiocytes.
Exisional biopsy of the lymph nodes were reported to be infiltrated with large histiocytes, lymphocytes, and plasma cells. Since the
patient was diagnosed as CDI, pituitary MRI was performed. MRI
detected normal pituitary gland thickness and signal intensity, diffuse enhancement in dynamic-contrast images, loss of the characteristic hyperintensity of the neurohypophysis, bilateral cavernous
sinus infiltration and expansion, anterior epidural focal thickening
below C3 level. Besides, sclerotic lesions were detected on thoracic
vertebraes and sternum. The patient was diagnosed as Rosai Dorfman disease with multi-system involvement, affected organs being
neurohypophysis, bones and skin. Interferon alpha treatment was
planned.
20 - 23 October, 2016
Discussion: Out of the 126 cases of RDD with CNS involvement in
the literature, only 5 had CDI. So RDD should always be kept as a
differential diagnosis of CDI with or without multi system involvement.
Keywords: Rosai Dorfman Disease, Histiocytosis, Central Diabetes Insipidus
Picture 1. Facial lesions
50
Antalya - Turkey
2016
O – 41
Neuroendocrine Tumors
Localization of an insulinoma by selective
intraarterial calcium stimulation with
hepatic venous sampling
Nafiye Helvacı, Bülent Okan Yıldız, Gökhan Baykal
Hacettepe University School of Medicine, Division of Endocrinology and Metabolism , ANKARA, TURKEY
Introduction: Insulinomas are rare neuroendocrine tumors characterized by excessive insulin secretion. When these tumors come to
clinical attention, they are usually small, measuring less than 2 cm
in most of the cases. The only curative treatment modality is surgical
resection of the tumor. Despite advances in imaging techniques, preoperative localization of insulinomas may be challenging.
Case: A 52-year-old man was admitted to the endocrinology clinic
due to recurrent episodes of confusion, blurred vision, diaphoresis, and dizziness lasting for 6 months. Clinical symptoms were
most likely to occur after a missed meal during the day. He had
a weight gain of about 6 kg in the preceding 6 months. His prior medical history and family history were unremarkable. Physical and neurological examinations were normal. He underwent a
supervised 72-hour fasting test. After 10 hours of fasting, the patient developed hypoglycemic symptoms and blood tests revealed
a low plasma glucose (42 mg/dL) and abnormally elevated levels
of insulin (40.4 µIU/mL) and c-peptide (3.13 ng/mL). Tumor localization studies including magnetic resonance imaging of the abdomen and endoscopic ultrasonography were negative. In order
20 - 23 October, 2016
to localize the tumor, selective intraarterial calcium stimulation
with hepatic venous sampling was performed. Calcium injection of
proximal splenic artery caused more than 2-fold increase in hepatic insulin above baseline, indicating a lesion at the body/tail region
of the pancreas. The patient underwent laparotomy. Careful manual palpation of the pancreas revealed a 1.5 cm nodular lesion in
the tail. Distal pancreatectomy was performed. Histopathological
findings were consistent with well-differentiated neuroendocrine
tumor showing positive staining with insulin, synaptophysin, and
chromogranin. Postoperatively, the patient was free of all previous
symptoms and discharged with normal glycemic values.
Conclusion: Selective intraarterial calcium injection of the major
pancreatic arteries with hepatic venous sampling is significantly
superior than the non-invasive imaging modalities as a preoperative localizing study for insulinomas. Since it is an invasive study,
must be reserved for the cases in which conventional non-invasive
imaging methods do not localize the tumor.
Keywords: insulinoma, intraarterial calcium stimulation, magnetic resonance
51
Antalya - Turkey
2016
0 – 42
Neuroendocrine Tumors
Known syndrome, a new case
Meltem Koca1, Seda Oğuz2, Derya Karakoç3, Uğur Ünlütürk2
1 Department of Internal Medicine, Hacettepe University, Ankara, Turkey
2 Division of Endocrinology and Metabolism, Hacettepe University, Ankara, Turkey
3 Department of General Surgery, Hacettepe University, Ankara, Turkey
Introduction and Background: MEN type 1 is a rare, autosomal
dominantly inherited syndrome presenting with various neoplasms
of the parathyroid glands, pancreas and anterior pituitary. Many
other endocrine and non-endocrine tumors also may complicate
the clinical picture.
Clinical Case: We report the case of a 29-year-old male who presented with an episode of hypoglycemia to an outside hospital. As
the prolonged supervised fasting test was positive; insulinoma was
clinically suspected. A subsequent abdominal magnetic resonance
imaging (MRI) study revealed two focal pancreatic mass lesions locating in the body and uncinate process of pancreas, measuring 22
mm and 13mm, respectively. Additional laboratory tests also revealed associating hypercalcemia and hyperprolactinemia. He was
referred to our center with the preliminary diagnosis of MEN type 1.
Primary hyperparathyroidism was diagnosed with clinical and laboratory findings and a subsequent pituitary MRI study depicted a
macroadenoma with a size of 10x9x11 mm. With these findings, the
20 - 23 October, 2016
patient underwent total pancreaticoduodenectomy as the patient
had a history of severe hypoglycemia. Pathological findings of the
surgical specimen confirmed the clinical diagnosis of insulinoma
by demonstrating multicentric well-differentiated foci of neuroendocrine tumors. These tumors stained positive for insulin and synaptophysin as well as negative for glucagon, calcitonin, S100 and
pan-keratin. Subtotal parathyroidectomy was also performed with
the final diagnosis of parathyroid hyperplasia. As for hyperprolactinemia treatment, we started oral cabergoline.
Conclusion: MEN type 1 is a rare, incurable but managable syndrome. Treatment plan depends on the patients’ dominant symptom. As the main symptom in our case was hypoglycemia, we elected to proceed with treatment of the pancreatic neuroendocrine
tumors before the removal of the parathyroid adenoma.
Keywords: MEN 1, insulinoma, hypoglycemia, pancreatic neuroendocrine tumor, hyperparathyroidism
52
Antalya - Turkey
2016
POSTER
PRESENTATIONS
20 - 23 October, 2016
53
Antalya - Turkey
2016
P – 01
Adrenal
Autoimmune Polyglandular Syndrome type 2in childhood
Afruz Mammadova, Miyase Bayraktar
Department of Internal Medicine, Ankara, Turkey
Introduction: Autoimmune Polyglandular Syndromes (APS) are
characterized by loss of immune tolerance to self-antigens leading
to autoimmune destruction of endocrine glands and other tissues.
Clinical Case: We are presenting the case of 28 years old female
patient. When the patient was 13 years old, she was referred to endocrinology department because of increased pigmentation of skin
and mucosal surfaces. Since physical and laboratory findings were
compatible with adrenal insufficiency, she was started on hydrocortisone treatment. The patient was also diagnosed Hashimoto’s
thyroiditis concurrently with adrenal insufficiency. She had a family history of hypothyrodism and diabetes mellitus type 2 (mother
and aunts). After six years she was admitted to our center with the
complaints of polydipsia, polyuria and loss of weight. She was diagnosed with type 1 diabetes mellitus. Since she had complaints
20 - 23 October, 2016
of abdominal pain and diarrhea, blood tests for Celiac disease antibodies were done, but was found negative and the colonoscopy
was normal. Autoimmune thyroid disease, autoimmune adrenal
insufficiency and type 1 diabetes mellitus supported the diagnosis
of autoimmune polyglandular syndrome type 2.
Conclusion: APS type 2 is the most common of the endocrinopathy syndromes. APS is characterized by autoimmune adrenal insufficiency in combination with autoimmune thyroid disease and/
or type 1 diabetes mellitus. APS type 2 is diagnosed primarily in
adulthood, usually in third or forth decades of life and is extremely
rare in childhood.
Keywords: Autoimmune adrenal insufficiency, diabetes mellitus
type -1, Hashimoto’s thyroiditis
54
Antalya - Turkey
2016
P -02
Adrenal
Ga-68 DOTATATE can provide surgery more
feasible in patient with devastating
pheachromocytoma mass.
Ayten Eraydın1, Zeynel Abidin SayIner1, Umut Elboğa2, Suzan Tabur1, Mesut Özkaya1,
Ersin Akarsu1, Mustafa Araz1
1 Department of Endocrinology and Metabolism, Gaziantep University, Gaziantep, Turkey
2 Department of Nuclear Medicine, Gaziantep University Hospital, Gaziantep, Turkey.
Objective: Pheochromocytomas are rare and if missed they can
expand devastating size. At this point bigger size can be a problem
for surgery. To know the mass’ nature and anatomic localization
clearly can help the clinician before the surgery and it can provide
optimal perioperative care for the patient. Ga-68 DOTATE labeled
somatostatin analogues to image neuroendocrine cancers has
raised the interests to use for NETs. Ga 68-DOTATATE PET-CT has
demonstrated superiority in lesion detection compared to scintigraphy and MRI. Consequently usage of Ga-68 DOTATATE PET-CT for
pheochromocytomas is a good option to demonstrate the mass and
it should be kept in mind as a tool to clarify the exact localization
and anatomy of the lesion.
Keywords: GA-68 Dotatate scan, pheachromocytoma, Adrenal surgery
Figure 1
Case: A 61-year-old male patient was admitted to surgery clinic
complaining of stomach ache. After physical evaluation and routine
blood tests surgeon performed computer tomography of abdomen.
They found out adrenal mass and they referred the patient to our
endocrinology outpatient clinic. We evaluated whether the tumor is
hormone secreting or not. We checked the patients complaints in
more detail and we realized that patient had hypertension, fatigue,
palpitation and flushing. His normetanefrine was 1221 mg/24h and
noradrenaline 318 mg/24/h. After biochemical evaluation patient
had diagnosis of pheochromocytoma. We decided to perform surgical intervention for the patient. But her adrenal mass had 10x12 cm
in diameter. Also the patient had several comorbid problems like
coronary heart disease and type 2 diabetes mellitus. Surgery would
be very life threatening for the patient. After further evaluation of
computer tomography we decided to use Gallium-68 Dotatate positron emission tomography technic before surgery.
Conclusion: Our aim was to reveal the mass’ anatomic position and
understand clearly the nature of the mass. Afer Gallium-68 Dotatate Pet scanning we realized that the mass has hypometabolic
region in the center. Also it had hypermetabolic region just only on
the surface of the mass. (Figure 1 ) After this information surgery
became more feasible. In this case we wanted to emphasize that if
a patient has an adrenal mass and surgery is life threating, Ga-68
Dotatate Pet scanning can be an useful technic to understand he
nature of the mass clearly.
20 - 23 October, 2016
Figure 1.Adrenal mass with necrosis in the center
55
Antalya - Turkey
2016
P – 03
Adrenal
Adrenal vascular cyst: A rare form of
adrenal mass
Javanshir Vahabov1, Seda OĞuz2, BÜlent Okan Yıldız2
1 Department of Internal Diseases, Hacettepe University, Ankara, Turkey
2 Department of Internal Diseases Endocrinology Subdivision, Hacettepe University, Ankara, Turkey
Introduction: Adrenal cystic lesions (CALs) are rare and form a
heterogeneous group of lesions. Adrenal vascular cysts account for
84% of adrenal cysts. These lesions are more common in women
and present clinically with abdominal pain or are found incidentally.
Since their imaging features are not specific, endocrine evaluation
should be performed carefully.
Case Presentation: 50-years-old woman was referred to our institution from a clinic where she presented with right sided flank
pain and abdominal ultrasound detected a nonspecific cystic lesion at the upper region of the abdomen suggesting a liver cyst.
An abdominal MRI showed a mass lesion of 43x22 mm at right adrenal gland which was hyperintense in T2A sequences, containing
calcified areas and showing marked contrast uptake. The characteristics of the lesion indicated preliminary diagnosis of pheochromocytoma or malignant adrenal mass rather than a benign lesion.
The hormonal evaluation was completely normal. The mass was
laparoscopically removed. Pathological examination revealed the
diagnosis of vascular cyst with positive CD 34 and CD 31 staining.
Figure 1. MRI
Conclusion: Adrenal cysts are rare but important because of the
clinical emergencies they may cause and the incidental malignancies or functioning tumors they may contain. Accordingly, all CALs
require complete endocrinological evaluation.
Keywords: Adrenal vascular cyst, adrenal mass, vascular cyst,
Figure 2. MRI
20 - 23 October, 2016
56
Antalya - Turkey
2016
P – 05
Bone/Calcium
A Fahr’s Syndrome Patient with Resistant
Hyperphosphatemia
İffet Dağdelen Duran
Department of Endocrinology and Metabolic Diseases, Kırıkkale Yüksek İhtisas Hospital, Kırıkkale, Turkey
Introduction: Fahr’s syndrome is a rare neurological disorder
characterized by abnormal calcified deposits in basal ganglia and
cerebral cortex. Clinical manifestations are neuropsychiatric abnormalities and movement disorders. There are various etiological
factors. Biochemical abnormalities are nearly always absent and
may worsen the course of the disease if untreated. We presented a
patient with primary hypoparathyroidism and Fahr’s syndrome who
has resistant hyperphosphatemia.
Case: A 74-year old woman admitted to our clinic with involuntary
movements and speech impairment. She had no disease or operation previously, or no disease in her family history. Her physical
examination was normal except chorea in right forearm, tremor
in both hands with orofacial dyskinesia and slurred speech. Her
blood test results were: Creatinine:0.76 mg/dl, Calcium (Ca):4.36
mg/dl, Phosphorus (P): 5.91 mg/dl, Magnesium (Mg): 1.79 mg/dl,
Parathormone (PTH):1.5 pg/ml, 25-OH-Vitamin D3 (25-OH-D):<3
ng/ml. Primary hypoparathyroidism was diagnosed and she was
hospitalized and treatment with parenteral calcium, and vitamin D
replacement continued with oral calcium carbonate and calcitriol.
To rule out cerebrovascular pathology, cranial computed tomography (CT) was performed and multiple bilateral symmetric calcifications were assessed in basal ganglia, periventricular regions
and both cerebellar hemispheres (figure1). This finding suggested
Fahr’s syndrome. Electroencephalography revealed pathological
pattern which necessitated 1000 mg/day sodium valproate to start.
Her neurological complaints did not change with recovery of calcium level. Haloperidol was added to resolve the unintentional movements. She was discharged calling her for the next visit. Ca:9.1 mg/
dl, P: 6.4 mg/dl, Mg: 1.9 mg/dl was retrieved after two weeks. After
one week the result of Ca:8.5 mg/dl, P: 6.5 mg/dl directed us to
re-hospitalize her to control her adherence to drug. Calcium citrate
dosage increased up to 9000 mg/day throughout hospital stay with
cessation of calcitriol and vit D3. This gave the results of Ca:8.5 mg/
dl and P: 6.3 mg/dl. Disability of hyperphosphatemia management
accompanied continuation of chorea and orofacial dyskinesia.
20 - 23 October, 2016
Conclusion: Hyperphosphatemia would accompany Fahr’s syndrome when the etiology is hypoparathyroidism. Seizures and
movement disorders in Fahr’s syndrome can be resolved with the
correction of phosphate and calcium levels. Despite managing normocalcemia, inability in normalization of phosphate levels would
be one reason not to establish stabilizing neurological manifestations of the syndrome.
Keywords: Fahr’s syndrome, hypoparathyroidism, hyperphosphatemia
Figure 1
Figure 1. Calcificatiton in basal ganglia
Figure 2
Figure 2. Calcification in cerebellar hemispheres
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2016
P – 08
Diabetes
Diabetes and Hypogonadism: Think of
Haemochromatosis!
Faisal Hamza Dalvi1, Mazhar Hamja Dalvi2
1 Aster Medical Centre, Dubai, United Arab Emirates
2 Department of Diabetes and Endocrinolgy,Al Noor Hospital,Abu dhabi,United Arab Emirates
40-year-old male was referred to the diabetes clinic for help in
managing his diabetes. Two years before his visit, diabetes was diagnosed after history of osmotic symptoms of polyuria, polydypsia
and weight loss. He was started on oral hypoglycaemic agents. He
initially responded to this treatment, but over the next one year his
medication doses were slowly raised until he was on 60mg of gliclazide and 2,000 mg metformin. At the time of referral, his fasting
blood glucose levels were in the range of 180 mg/dl and and his
haemoglobin A1c (A1C) was 10%. He was started on basal insulin,
insulin glargine.
His medical history was not significant for any alcohol intake or
hepatitis. Family history was negative for diabetes and hemochromatosis. His review of systems was positive for joint discomfort
in both hands along with 6 month history of decreased libido and
erectile dysfunction.
Physical exam revealed normal vital signs and no retinopathy or
other signs of diabetic complications. His hand joints showed mild
swelling and tenderness over the proximal interphalangeal joints,
and his skin was slightly, diffusely hyperpigmented.
Lab data included a random glucose of 253 mg/dl, A1C of 10%,
normal creatinine and electrolytes, aspartate aminotransferase
(SGOT) of 77 IU/L (normal < 44 IU/L), alanine aminotransferase
(ALT) of 144 IU/L (normal < 31 IU/L ), normal bilirubin and alkaline
phosphatase levels, low testosterone level 9 nmol/l (normal range
20 - 23 October, 2016
12-30 nmol/l), and negative hepatitis antigen screen. His iron level
was 386 μg/dl (normal < 155 μg/dl) with an iron-binding capacity of
296 μg/dl (normal < 400) and percent transferrin saturation of 97%
(normal < 50%). Serum ferritin was 3028 μg/l (normal < 160 μg/l).
Screen for autoimmune hepatitis was negative. Genetic testing
demonstrated homozygosity for the C282Y chromosome. Referral
to the hepatology clinic was done and liver biopsy was carried out,
which identified increased iron stores and early periportal fibrosis.
A detailed review of the liver functions done prior to onset of diabetes was done which revealed SGOT and ALT always in higher range
which was always thought to be fatty liver related, but looking back
patient had haemochromatosis from the begining and diabetes was
secondary to iron deposition in pancreas.
Following confirmation of a diagnosis of hemochromatosis, he
was started on venesection. Family screening was encouraged and
resulted in the finding of asymptomatic diabetes associated with
hemochromatosis in his brother. The blood sugars improved on insulin and A1C improved to 7%. The patient was found to have hypogonadotrophic-hypogonadism (loss of libido, erectile dysfunction
and low testosterone) secondary to his haemochromatosis. He was
started on six-weekly testosterone injections with good effect. He
also had an MRI scan of his pituitary which was normal.
Keywords: Diabetes, Hypogonadism,Haemochromatosis
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2016
P – 14
Diabetes
Increased fastıng and stimulated c- peptide
levels after the diagnosis of type 1 diabetes
Gonca Tamer
Medeniyet University, Göztepe Training and Research Hospital, istanbul, turkey
Context: Recently a few studies showing ability of a cell to differentiate into insulin–producing β-cells after prolonged duration of
diabetes and reconstruction of new insulin–producing cells in the
absence of autoimmunity in mice with completely ablated β-cells
were reported. Here, we present a patient with type 1 diabetes and
increased fasting and mixed-meal stimulated C-peptide levels 10
years after the diagnosis.
Case Description: A female patient with type 1 diabetes for 10
years was referred to our endocrinology clinic for hypoglycemia at-
20 - 23 October, 2016
tacks. Her fasting glucose, HbA1C, fasting and mixed-meal stimulated C-peptide levels were measured three years ago and recently.
Fasting and mixed-meal stimulated C-peptide levels were found to
be increased compared to the levels of three years ago (<0.01 ng/
mL vs. 0.06 ng/mL and <0.01 ng/mL vs 0.28 ng/mL respectively).
Conclusion: It is possible that β-cell function may increase in type
1 diabetes, a decade after the diagnosis as observed in our patient.
Keywords: type 1 diabetes, β-cell, mixed-meal stimulated C-peptide
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2016
P – 18
Female Reproductive Endocrinology
Is prior pregnancy a key point for
replacement therapy decision for current
pregnancy in patients with Gaucher disease?
Zeynel Abidin SayIner, Ayten EraydIn, Mesut Özkaya, Ersin Akarsu, Suzan Tabur,
Mustafa Araz
Department of Endocrinology and Metabolism, Gaziantep University, Gaziantep, Turkey
Background: Disease (GD) is characterized by deficient acid b-glucosidase activity and it is the most common lysosomal storage
disorder. Recent articles showed that women with GD can have
successful pregancies with or without replacement thrapies. Patient’s informed decision to continue or not for treatment should
be respected. Aside from this clinicans need keypoint to be sure
for continuing or discontinuing the therapy during the pregnancy.
According to our two cases we realized challenges that occurred
throughout prior pregnancy can be a waypoint for the current pregnancy. Despite the lack of supporting studies, it may be advisable
to consider prior events during previous pregnancies that can illuminate clinicians about continuing or discontinuing the treatment
throughout current pregnancy. Cases: Our patients are women and
they have type 1 Gaucher disease (GD).
Patient 1 was diagnosed at age 15 years due to abdominal enlargement and bone pain. She had been treated with imiglucerase (30 U/
kg every 2 weeks) since 23 years old. At age 23 she became pregnant. This was her second pregnancy. Her first pregnancy was an
uncontrolled pregnancy and she did not take replacement therapy,
she experienced severe bleeding problems. After this uncontrolled
and drug free period we started imiglucerase therapy to the patient. After 1 year of replacement therapy she got pregnant again.
She continued replacement therapy during her pregnancy with the
same dose. There was no additional laboratory parameter change.
Her clinical state was stable and there was no unexpected event
20 - 23 October, 2016
during her pregnancy. The patient delivered a male infant with
elective cesarean section. The infant’s birth vital parameters were
good and there was no additional health problem with the infant.
Patient 2 was 33 years old. She underwent elective splenectomy at
age 26 years old. She was treated with 20-30 units/kg of imiglucerase every two weeks. She was diagnosed with severe thrombocytopenia, bone pain, fatigue and splenomegaly. She became pregnant
at age 32 years and with informed consent she discontinued her replacement therapy throughout all pregnancies. This was her third
pregnancy and she discontinued replacement therapy during her
previous pregnancies. According to patient assertion there was no
health problem during both 2 pregnancies and deliveries. A conservative approach with close monitoring of mother and baby was
done during the pregnancy. In the 38th week of pregnancy a healthy
male infant was delivered with cesarean section. There was an uneventful delivery and both mother and infant were healthy.
Conclusion: In conclusion we herein present two successful pregnancies and breastfeeding period in female patient with Gaucher
disease. We believe that prior experienced events during the previous pregnancies should be a good waypoint to predict the risk of
undesirable events throughout the current pregnancy and breastfeeding.
Keywords: Gaucher disease, enzyme replacement therapy, pregnancy
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2016
P – 19
Neuroendocrine Tumors
A men1 case presented with
hyperinsulinemic hypoglycemia
Dilek Karakaya ArpacI1, Mustafa Ünal1, Hande Oran2, İlker Öz3, Taner BayraktaroĞlu1
1 Division of Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine, Bulent
Ecevit University, Zonguldak, Turkey
2 Department of Radiology, Faculty of Medicine, Bulent Ecevit University, Zonguldak, Turkey
3 Department of Internal Medicine, Faculty of Medicine, Bulent Ecevit University, Zonguldak, Turkey
Introduction: Multiple Endocrine Neoplasia-1 (MEN-1) is a rare
syndrome (1/30000) which has autosomal dominant genetic transmission but sporadical occurrence is also reported. We report
herein a MEN-1 case presented with hyperinsulinemic hypoglycemia diagnosed both hyperparathyroidism and asymptomatic hyperprolactinemia.
Case: A twenty-eight years old man had been suffering with frequent hunger symptoms for five-six years. When he was admitted to emergency room with complaints of perspiration and loss of
consciousness, his blood glucose was found 35 mg/dl, after dextrose infusion his glucose was improved. His blood pressure was
120/75 mmHg, heart rate was 78 /min.; his height and weight were
180 cm and 108 kg respectively, with body mass index of 33.3 kg/m2
on admission. In prolonged fasting hypoglycemia test, at 18 hours
of prolonged supervised fasting test (Table 1) his blood glucose was
38 mg/dl; insulin was 10,83 uIU/mL. C-peptide was 1,51 ng/mL,
urinary ketone was also negative. The MR imaging revealed two
mass lesions that the first was sized 23x20 mm lobulated counter (border) in level of pancreatic body –tail section and the second
one was approximately sized 13 mm in uncinate process. Because
of the patient’s history of nephrolithiasis; hypercalcemia and hyperparathyroidism were observed (Table 2) Primary hyperparathyroidism was considered as a part of MEN-1. Bilaterally parathyroid
adenomas were observed in parathyroid ultrasonography (figure
3 and 4) and scintigraphy. There were regular hypoechoic nodular
lesions suspicious of parathyroid adenomas that are 6,5*9,1*13,1
mm sized in left side and 13,6*11,3*16 mm sized in right side.
Parathyroid washout results were respectively 3482 pg/dL in right
lesion and 2149 pg/dL in left lession.
Our patient had both insulinoma and primary hyperparathyroidism
so that we checked anterior pituitary hormones and we found that
prolactin level was high (205 ng/mL). After that pituitary MRI was
revealed a macroadenoma sized 14*12 mm extending to the suprasellar cistern, isointense in T1 section and heterogenous enhancing in T2 section. Visual field was normal. According to clinical, radiological and hormonal results; the diagnosis of Multiple
Endocrine Neoplasia -1 (MEN-1)-Wermer syndrome was verified.
20 - 23 October, 2016
Our patients underwent total pancreatectomy for insulinomas, follow up parathyroid adenomectomies. Also cabergoline was administered for his prolactinomas. He is referred to genetic analyses.
Discussion: MEN 1 syndrome is characterized with primary hyperparathyroidism (90%); pancreatic neuroendocrine tumors (75%)
and pituitary adenomas (35%) In our patient multiple insulinoma
lesions were detected as first presentantion, in addition as a part
of MEN-1 syndrome, bilateral parathyroid adenomas and prolactinoma were diagnosed concomitantly. According to our case, we
suggest that clinical expression of the MEN-1 syndrome is variable,
and its components can be diagnosed simultaneously with careful
examinations.
Keywords: MEN1, insulinoma, primary hyperparathyroidism, prolactinoma
Figure 1. PANCREAS MRI
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2016
Figure 2. Pancreas MRI
Figure 5. Pituitary MRI
Table-1 Results of the prolonged fasting test
Results
0.
12. h.
18.h.
Glucose (mg/dL) (74-100)
107
50
38
Insulin (uIU/mL) (3-25)
65,76
10,91
10,83
C-peptide (ng/mL) (0.9-7.1)
9,33
2,09
1,51
Table 2.Baseline laboratorial results
Figure 3. Parathyroid USG
Parameters
Results
ParathormonH (pg/mL) (15-65)
133,9
Alkalen phosphatase (U/L)(30-120)
118
Calcium (mg/dL) (8.5-10.5)
12,7
Phosphorus (mg/dL) (2,5-4,5)
2,7
Albumin (g/dL) (3,5-5,2)
5,1
25 OH D vitamin (ng/mL.) (30-100)
10,3
24-hour urinary calcium (mg/a day) (100-300)
532
Creatinine clearance (mL/ minute)
139
Prolactin (ng/mL)
205
Prolactin levels after precipitated with PEG (ng/mL)
289
Follicule Stimulating Hormone (m IU /m L)
1,74
Luteinizing Hormone (m IU /m L)
2.47
Total testosterone (ng/m L) (1,75-7,81)
2,28
Cortisol (µ IU/mL) (3.7-19.4)
19
Adrenocorticotropin (pg/mL) (10-46)
70,7
Throid Stimulating Hormone (µ IU/mL) (0.33-4.2)
1,37
Growth Hormone (ng/mL) (0.05-8.6)
0,35
Figure 4. PARATHYROID USG
20 - 23 October, 2016
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2016
P – 20
Neuroendocrine Tumors
The localization of the occult insulinoma
by selective arterial calcium stimulation
test in a patient with persistent
hyperinsulinemic hypoglycemia
Mustafa Ünal1, Dilek ArpacI1, İlker Öz2, Ece Konuk3, Taner BayraktaroĞlu1
1 Division of Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine, Bulent
Ecevit University, Zonguldak, Turkey
2 Department of Radiology, Faculty of Medicine, Bulent Ecevit University, Zonguldak, Turkey
3 Department of Internal Medicine, Faculty of Medicine, Bulent Ecevit University, Zonguldak, Turkey
Introduction: Persistent hyperinsulinemic hypoglycemia is caused
commonly by insulinoma or by nesidioblastosis. This disease is
characterized by persistent hypoglycemia as a result of hypersecretion of insulin from insulinoma or β-cell hyperplasia of the pancreas in adults representing 0.5%-5% of cases of organic hyperinsulinemia.
We report a very rare case with persistent hyperinsulinemic hypoglycemia due to hypersecretion of insulin from β-cell of the pancreas.
Case: A 61-year-old woman was referred to our center because
of persistent and repeated severe hypoglycemia. Her past history
included the diagnosis of essential hypertension treated vith nebivolol 5 mg per day and recovered facial paralysis. The physical examination revaled the following: height 168cm, weight 80 kg, blood
pressure 120/70 mmHg, pulse 67/minutes. No other signs were
detected in her system examinations. Hyperinsulinemic hypoglycaemia was diagnosed with insulin level >300 uIu/ml and C peptide
level 13.50 nmol/l during 35 mg/ml fasting blood glucose. Imaging modalities and laboratory examination, including repeated CT,
MRI scans and endosonography were negative. Subsequent testing
involved stimulation of the pancreas through cannulation of the
pancreatic vascular supply by interventional radiology. Selective
arterial calcium stimulation test revealed marked insulin release
to calcium challenge across multiple pancreatic segments (Table
1). Insulinoma was not visualized by angioraphy during stimulaton
test. On the follow up, the patient rejected the operation, and was
treated with somatostatin analogs and diazoxide.
Conclusion: Once the diagnosis of hyperinsulinemic hypoglycaemia was made, patients underwent preoperative localization by US,
CT and/or MRI. One or several non-invasive radiological imaging
procedures and selective arterial calcium stimulation test were
performed, to referred patients with hyperinsulinemic hypoglycaemia after a negative endosonographical localization of insulinomas.
Selective arterial calcium stimulation test and endosonography of
pancreas are complementary procedures and sometimes may be
coupled to reach maximal diagnostic performance for localization
of the tumor.
Keywords: hyperinsulinemic hypoglycemia, insulinoma,nesidioblastosis
Table 1. Selective arterial calcium stimulation test (SACT)
Time (Seconds
Proximal splenic artery
Superior mesenteric artery
Gastroduodenal artery
Hepatic artery
Intrahepatic artery
0.
30.
60.
90.
120.
180.
300.
Insulin (uIU/ml)
1228
1252
1336
1257
1285
1243
98
Glucose (mg/dl)
176
174
176
177
176
177
165
Insulin (uIU/ml)
1380
1264
1330
1338
1349
1333
1370
Glucose (mg/dl)
178
178
174
177
172
179
180
Insulin (uIU/ml)
1096
1171
1210
>300
>300
297
21
Glucose (mg/dl)
173
170
170
174
169
172
170
Insulin (uIU/ml)
1098
1091
1112
1274
1334
1120
1049
Glucose (mg/dl)
155
150
158
154
174
179
177
Insulin (uIU/ml)
1241
1302
1288
1274
1334
1287
1260
Glucose (mg/dl)
175
177
178
177
174
179
177
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P – 21
Neuroendocrine Tumors
A case report: Biochemically and clinically
persistent medullary thyroid carcinoma
for eight years
Şerife Nur Boysan1, Güzide Özdil2, Emel Günay3, Önder Peker4, Mete Düren5,
Mustafa Sait Gönen6
1 Istanbul Bilim University, Endocrinology, Istanbul, Turkey
2 Istanbul Bilim University, Radiology, Istanbul, Turkey
3 Istanbul, Bilim University, Nuclear Medicine, Istanbul, Turkey
4 American Hospital, Istanbul, Turkey
5 Istanbul University,Cerrahpasa Medical Faculty, Istanbul, Turkey
6 Istanbul Bilim University, Endocrinology, Istanbul, Turkey
Introduction: Though medullary thyroid carcinoma (MTC) accounts
for 5 to 10 % of all thyroid cancers. the prognosis is worse than
differiantiated thyroid carcinoma. It was reported as 10-year survival of 40-90 %. The hereditary form is 25 % and sporadic form is
75%. The variable clinical course of patients was reported because
of a relatively slow tumour growth but early lymphatic metastatic
spread. We are presenting a patient with asymptomatic but biochemically active and clinically persistent MTC.
Clinical Case: Diagnostic evaluation and treatment
A 40-year old man presenting with a palpable thyroid nodule. Ultrasonography showed calcific 18 mm nodule in the left lobe and
calcific 30 mm lymph node in the left juguler. Cytologic examination revealed MTC both in thyroid and lymph node in tle left side.
Total thyroidectomy and lymph node dissection showed multifocal
(2.5 cm in the left lobe and 0.6 cm in the right lobe) with lymphovascular and locoregional invasion of MTC but also positive mediastinal lymph node metastasis. Postoperative serum calcitonin
was 352 pg/m L and whole body scanning withTc-99M V-DMSA revealed pathologic accumulation on the left side of neck. After three
months he was reoperated as sternotomy with bilateral lymph
node dissection and serum calcitonin was decreased to 83 pg/mL.
He was asymptomatic and urinary catecholamines were normal
and showed no hepatic metastasis with computed tomography.
Follow-Up: After seven years he was referred for control. Serum
calcitonin was 104 pg/mL and US showed supraclavicular solid masses (22 and 19 mm) with submandibular pathologic lymph
node (11 mm) in the left side. Positron emission tomography (PET)
using 18F-FDG confirmed hypermetabolic nodular lesion. Cyotologic examination from lymph node was non diagnostic.
After six months serum calcitonin was 108 pg/mL. The volumetric magnetic resonance imaging showed bilateral multiple cervical
lymph nodes (the largest was 13 mm in the right side) and anterior
prevertebral soft tissue lesion in the right side at the level of 1 st
intercostal space (17 mm) and two lymph nodes in the right paratracheal region at the level of upper mediastinum. The incisional
biopsy showed connective tissue. PET using(18F-DOPA) revealed
pathologic accumulation.
We suggested to the patient to test for Rearranged during transfection (RET) gene but he did not want to do.
Conclusion: MTC shows slow tumour growth with early lymphatic metastasis and postoperative increased serum calcitonin levels
suggests persisting tumour and different imaging techniques reveal clinically persistent disease. Age at diagnosis, tumour stage
and genetic analysis are important factors in determining prognosis. We suggested the third operation to our patient considering a
prolonged indolent course of disease even with metastasis.
Keywords: medullary, thyroid,carcinoma, persistent, disease
20 - 23 October, 2016
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2016
P – 22
Pituitary
May headache pattern be a clue for
differential diagnosis in patients with
pituitary mass?
Zeynel Abidin Sayıner1, Ayten Eraydın1, İbrahim Erkutlu2, Ersin Akarsu1, Mustafa Araz1
1 Department of Endocrinology and Metabolism, Gaziantep University, Gaziantep, Turkey
2 Department of Neurosurgery, Gaziantep University, Gaziantep, Turkey
Objective: Intrasellar aneurysms originated from the cavernous
carotid are rare. This type of aneurysms can simulate pititary adenomas. It is crucial to confirm an aneurysms suspicion with conventional angiography before surgery for a pituitary mass. There
is not always a relationship between headaches and pituitary adenomas. Therefore, the treatment of pituitary tumors does not always result in resolution of headaches. Besides headache is not a
surgery indication for surgery. But if headache pattern is severe or
unstable detailed evalution of pituitary region should be done.
Case: A 45-year-old female patient was admitted to neurology outpatient clinic complaining of headache and fatigue. Neurologist
performed cranial magnetic resonance imaging. They found out pituitary adenoma and they referred the patient to our endocrinology
outpatient clinic. Pituitary adenoma was in the supraselar region
and it was located near internal carotid artery. It was 13x14 mm in
diameter. We evaluated the patients whether the tumor is hormone
secreting or not. We did not find any sign of hormone secretion in
physical examination and biochemical tests. Patient has no other
symptom except from the headache. It was thought that the patient
had non secreting pituitary macro adenoma. The patient’s headache was getting worse during the evaluation. We hospitalized the
patient and we reevaluated the MRI of the patient. There was round
shape adenoma but the border of the adenoma was very regular
and it was like a coin shape. Moreover, it had low signal on MRI.
(Figure 1) We decided to perform 3 dimensional computer tomography of brain. (Figure 2) It showed us saccular aneurysm which is
originated from ophthalmic branch of internal carotid artery and it
was located in the supraselar region. Then we performed cerebral
angiography and confirmed the aneurysm.
Conclusion: Headache pattern be a clue for differential diagnosis
in patients with pituitary mass. As shown in our case there can be
different causes other than the adenomas. Patients who have severe headache or change in the headache pattern with pituitary adenoma make us think that there can be another reason aside from
adenoma such as aneurysms.
Figure 1. Pituitary adenoma with low signal on MRI
Figure 2. 3D Computed tomography angiography of the aneurysm
Keywords: Pituitary mass, headache, aneurysm
20 - 23 October, 2016
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2016
P – 23
Pituitary
Acromegaly And Mccune-Albright Syndrome:
Case Report
Dilek Yazıcı, Faruk Alagöl, Havva Sezer
Deparment of Internal Medicine, Koc University School of Medicine, Istanbul, Tukey
Introduction: McCune Albright Syndome (MAS) includes the classic triad of poly/monostotic fibrous dysplasia (FD), cafe-au-lait
spots and hyperfunctioning endocrinopathies (hyperthyroidism,
acromegaly and cushing’s syndrome). This syndrome is characterized by autonomous hyperfunction of variety of cells that respond
to extracellular signals through activation of the hormone sensitive
adenylate cyclase system, which catalyzes the production of cAMP.
Acromegaly affects around 20% of MAS patients. We report here a
patient with MAS and acromegaly.
Case: A 53-year old women presented with sweating, palpitation
and neck swelling at 38 years old in 2001. She was diagnosed with
bone fibrous dysplasia of the skull, which was polyostotic at 4 years
old. She had short stature and precocious puberty. The patient was
hyperthyroid and uptake was increased at the right side of the thyroid. Thyroid ultrasonography revealed many nodules, the greatest
being 38x30 mm in the right lobe. Fine needle aspiration cytology was benign. Radioactive iodine (RAI) therapy was given in 2002.
After RAI, the patient was hypothyroid and levothyroxine therapy
was begun. Hypertension, fatty liver disease and diabetes mellitus
developed in 2012. The craniofacial fibrous dysplasia lesions grew
and serum growth hormone level was 7.2 ng/mL and IGF-1 was 979
ng/L (94-252). Serum growth hormone level was not suppressed by
oral glucose tolerance test.
pituitary. For these reasons, medical treatment is preferred in patients with MAS-associated acromegaly. SAs decrease GH/IGF-1
levels in most patients but do not achieve normal levels generally.
GH receptor antagonist pegvisomant is often effective. Malignant
transformation of fibrous dysplasia is possible due to irradiation of
bone lesions or GH/IGF-1 excess itself. Pituitary irradiation should
be avoided and diagnostic irradiation should be kept to a minimum.
Keywords: McCune Albright Syndome, Hyperthyroidism, Acromegaly, Cushing
Figure 1. Magnetic resonance imaging in coronal view of microadenoma (9 mm) in the left side of the pituitary gland surrounded by
cranial fibrous dysplasia with MAS and acromegaly
Octreotide 20 mg was begun and cabergoline was added. The medical therapy was ineffective even though the cabergoline (DA) dose
was increased up to 2 mg per week and octreotide (SA) dose was
up to 40 mg per month for 4 years of treatment. Surgical removal of
the pituitary adenoma was technically difficult, due to the thickness
of the cranial dysplasia at the skull base and the risk of hemorrhage given the high vascularity of fibrous dysplasia.
Conclusion: The diagnosis of acromegaly in a patient with MAS
may be delayed due to craniofacial fibrous dysplasia, masking the
dysmorphic craniofacial effects of acromegaly. Pituitary surgery
may be difficult or impossible in patients with MAS. Total hypophysectomy is generally required due to diffuse involvement of the
20 - 23 October, 2016
Figure 1. 2015 Hipofiz - T1-Coronal
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P – 24
Thyroid
Accurately localizing the thyroid tissue in
mature cystic teratoma of ovary by single
photon emission computerized tomography/
computerized tomography
Rıfkı Üçler1, Yusuf Demir2, İsmet Alkış3, Gülay Bulut4
1 Department of Endocrinology, Yüzüncü Yıl University, Van, Turkey
2 Department of Nuclear Medicine,Yüzüncü Yıl University, Van, Turkey
3 Department of Obstetrics and Gynecology, Yüzüncü Yıl University, Van, Turkey
4 Department of Pathology, Yüzüncü Yıl University, Van, Turkey
A 30 years old woman was using propylthiouracil for 3 years due to
hyperthyroidism. Although increased thyroid function was found,
the gland was normal in ultrasonography (USG). Abdominal USG
detected a cystic pelvic mass in left ovary. Magnetic resonance imaging showed 60 mm × 45 mm cystic mass in left ovary. Radioiodine uptake (10 μCi) and thyroid scintigraphy with 185 MBq (5
mCi) Tc99m were also normal. A whole body scan performed 48
h after oral ingestion of 29.6 MBq (0.8 mCi) iodine131 (I131) capsule, revealed a round structure located to the left lower abdomen.
Iodine uptake was detected in this cyst that was compatible with
functional thyroid tissue demonstrated by single photon emission
computerized tomography/computerized tomography (SPECT/CT)
[Figure 1]. Left salpingooophorectomy was done with frozen section. Histopathology confirmed mature cystic teratoma that was
containing thyroid follicles. The euthyroid state was achieved. Struma ovarii is a rare and usually a benign ovarian teratoma, which
consist of thyroid tissue in the ovarian mass. These tumors are
associated with hyperthyroid disease in 5–20%. Clinical features
may resemble ovarian malignancy and rarely can be metastatic.
Typically presents as a multicystic mass with lobulated surface and
thickened septic or cyst walls with some solid components. High
attenuation areas and calcifications in the solid components are
common findings on CT images. Scintigraphy with Tc99m pertechnetate, I123, and I131 were used for diagnosing a hyperfunctioning struma ovarii on the basis of higher uptake of the radionuclide
with planar images. If the clinicians or nuclear medicine physician
do not consider the extrathyroidal tissue as a result of hyperthyroidism, patient’s diagnosis cannot be made by the other nuclear
medicine test such as I131 uptake and thyroid scintigraphy. So, the
extrathyroidal cause of hyperthyroidism can lead to the dilemma
in nuclear medicine. In our patient, an I131thyroid uptake was in
normal ranges, and a Tcpertechnetate scan of the thyroid showed
normal distribution. I131 whole body scan identified the cause of
hyperthyroidism. Further, SPECT/CT localized the thyroid tissue in
large pelvic mass accurately.
20 - 23 October, 2016
Keywords: Hyperthyroidism, singlephoton emission computerized
tomography/computerized tomography, struma ovarii
Figure 1. Whole-body scan was performed 48 h after oral ingestion
of 29.6 MBq (0.8 mCi) I-131 sodium iodide capsule revealed a focal
uptake.
(a) Planar image shows focal iodine uptake located to the left
lower abdomen. In single-photon emission computerized tomography/computerized tomography images this focal uptake was
corresponding to the solid components of the large cyst which is
containing calcification (arrow). (b) Low dose unenhanced computerized tomography without intravenous contrast images. (c) Axial
fusion single-photon emission computerized tomography/computerized tomography images. (d) Coronal fusion single-photon emission computerized tomography/computerized tomography images
67
Antalya - Turkey
2016
P – 25
Thyroid
Propylthiouracil Seemingly Lower
Responsive Two Graves’ Patients
İffet Dağdelen Duran
Department of Endocrinology and Metabolic Diseases, Kırıkkale Yüksek İhtisas Hospital, Kırıkkale,Turkey
Introduction: Although methimazole (MMI) and propylthiouracil (PTU) have long been used to treat hyperthyroidism caused by
Graves’ disease (GD), there is a tendency to use MMI depending
on its lower hepatotoxicity and better treatment results. However
PTU would be addressed when MMI is unavailable, counting on its
comparable effect to MMI.
Case1: An 18 year-old girl was referred to our clinic with palpitations, weight loss and irritability. Her blood test results showed
Thyrotropin (TSH):0,00 µIU/ml, free T4 (fT4):3,54 (0,89-1,79) ng/dl,
free T3 (fT3):17,57 (2,3-4,2) pg/ml, anti-thyroid peroxidase:426 IU/
ml while thyroid ultrasonography(USG) revealed mild heterogeneity
with no nodule and thyroid 99m Technetium-pertecnatate scintigraphy showed diffuse increased uptake. GD was diagnosed and MMI
30 mg/day was started. After MMI was realized to be out of stock,
PTU 300 mg/day was prescribed. At second visit after one month
she had TSH: 0,00 µIU/ml, fT4:2,74 ng/dl, fT3:12,24 pg/ml which
heralded increasing the dosage to 450 mg/dl. Next time her blood
test retrieved much better results with fT4:1,87 ng/dl, fT3:6,74 pg/
ml. Since she was willing to have a tonsillectomy operation in recent time we used the same dosage. However, her hormone levels
was not normal at next visit that we increased PTU to 600mg/day.
One month later she had TSH: 0,00 µIU/ml, fT4:1.76 ng/dl, fT3: 7.12
pg/ml. MMI availability after that time allowed us to start MMI at
60 mg dosage. On 15 days of MMI fT3 and fT4 decreased to normal.
Waiting TSH to get lower, 40 mg MMI was used and normal TSH
20 - 23 October, 2016
with fT4:0.377 ng/dl and fT3:1.83 pg/ml was provided. This essentialized to lower the dosage further. On 10 mg MMI, euthyroidism
was managed and the patient underwent operation.
Case 2: A 50-year old man arrived at clinic with fatique and palpitations. His blood test results were: TSH:0.00 µIU/ml, fT4:3.6 ng/dl,
fT3:8.7 pg/ml. Thyroid USG and scintigraphy showed toxic diffuse
multinodular goitre. MMI 30 mg/day was started. Since unavailability of the drug PTU 300 mg/day was chosen. After one week
fT4:2.55 ng/dl, fT3:8.37 pg/ml were found. Under one month treatment results were:TSH:0.00 µIU/ml, fT4: 2.24 ng/dl, fT3: 7.95 pg/
ml. At same PTU dosage we continued with no alteration in hormone level and we increased the dosage to 450 mg/day. At next
visit the normalization of hormones could not be managed when
MMI rendered available. MMI 30 mg/d was used then. This yielded
TSH:0.30 µIU/ml, fT4:1.1 ng/dl, fT3:3.8 pg/ml at only one month of
therapy.
Conclusion: MMI should be used in every patient who chooses antithyroid drug therapy for GD except some occasions or when MMI
is unavailable. PTU is preferred then. However, PTU would not be
beneficial usually because of nonadherence of patient. PTU displacement with MMI would develop quicker and higher response,
probably because of more compliance of patient. Possible role of
pharmacodynamics of PTU already used might also be considered.
Keywords: Propylthiouracil, Response, Graves’
68
Antalya - Turkey
2016
INDEX
A
Ahsen, Hilal
48
Akarsu, Ersin
55, 60, 65
Akgün, Ayşegül
16
Akıncı, Barış
14, 18
Aksakal, Nihat
47
Akyıldız, Mahir
15
Alagöl, Faruk
66
Alkış, İsmet
67
Altunbaş, Hasan Ali
9, 21, 35
Altuntaş, Yüksel
40
Aral, Ferihan
47
Aral, Yalçın
29
Araz, Mustafa
55, 60, 65
Arpacı, Dilek Karakaya61, 63
Aytürk, Semra
45
Çolak Bozkurt, Nujen
29
Çuhacı, Neslihan
13, 48
D
Dağdelen Duran, İffet 24,
57, 68
Dağdelen, Selçuk
43, 46
Dalmatova, Anna
37
Dalvi, Faisal Hamza
58
Dalvi, Mazhar Hamja
58
Demir, Tevfik
14
Demir, Yusuf
67
Diri, Halit
18
Doğan, Özlem
9, 21, 35
Doğruel, Hakan
9
Doğrul, Ahmet Bülent
46
Düren, Mete
64
B
Bahçecioğlu, Adile Begüm
50
Bakıner, Okan
36, 38
Balcı, Mustafa Kemal 9, 21,
35
Başer, Hüsniye
48
Başpınar, Batuhan
25
Baykal, Gökhan
51
Bayraktar, Miyase
20, 54
Bayraktaroğlu, Taner61, 63
Berker, Dilek
17, 30
Beştepe, Nagihan
32
Bilen, Cenk Yücel
43
Bilginer, Cüneyt
13
Boysan, Şerife Nur
64
Bozkırlı, Emre
36, 38
Bozoğlan, Hümeyra
9, 21, 35
Bulut, Gülay
67
E
Ekşi Haydardedeoğlu, Filiz36, 38
Elboğa, Umut
55
Eraydın, Ayten
55, 60, 65
Erbaş, Tomris
43
Erdoğan, Can Emre
27
Erkutlu, İbrahim
65
Ersoy, Canan
11
Ersoy, Reyhan
13, 32, 48
Ersöz Gülçelik, Neşe24, 29
Ertan, Yeşim
10
Ertörer, Melek Eda
36, 38
Ertürk, Erdinç
11
Eyüpoğlu, Damla
26
F
FakI, Sevgül
Fırat, Sevde Nur
C
Cansu, Güven Barış
Cerit, Ethem Turgay
Culha, Cavit
44
23
29
Ç
Çakır, Bekir
13, 32, 48
Çalışkan Yıldırım, Eda
20
Çavdar, Ümit
14
Çetin, Zeynep
17, 30
13
29
G
Gönen, Mustafa Sait
Grineva, Elena
Güler, Serdar
Gül, Nurdan
Günay, Emel
Gürer, İnanç Elif
Gürlek, Alper
Güven, Deniz Can
20 - 23 October, 2016
69
64
37
17, 30
22
64
35
8, 50
8
H
Hacışahinoğulları, Hülya
22
Han, Ünsal
13
Helvacı, Nafiye
8, 20, 51
K
Kalayoğlu Beşışık, Sevgi
22
Karaca, Anara
29
Karagülle, Mustafa
44
Karakoç, Derya
52
Karcı, Alper Çağrı
17, 30
Kardaş, Rıza Can
46
Kardes, Özgür
36
Karonova, Tatiana
37
Kartal, İlkay
19
Kav, Taylan
25
Kayaselçuk, Fazilet
36
Kılıçarslan, Aydan
32
Kılıç, Mehmet
32, 48
Kıyıcı, Sinem
45
Klyaus, Natalia
37
Koca, Meltem
52
Koç, Gönül
29
Konuk, Ece
63
Korkmaz, Hakan
13
Kubat Üzüm, Ayşe
22, 47
Kuzu, İdris
40
M
Makay, Özer
Malay, Özer
Mammadova, Afruz
Mestanzade, Metban
Metin, Melike Ruşen
16
10
54
22
32
N
Nasıroğlu İmga, Narin17, 30
Nika, Christina
34
O
Oğuz, Seda
Omma, Tülay
Oran, Hande
Antalya - Turkey
8, 25, 43, 46, 52, 56
29
61
2016
Ö
S
Öğmen, Berna
13
Öğüt, Tahir Saygın
21, 35
Önder, Semen
47
Özdemir, Didem
32
Özdemir Kutbay, Nilüfer 15,
16, 18, 27
Özdemir, Murat
10
Özdil, Güzide
64
Özenmiş, Tahsin
29
Özgen, Gökhan
10, 15, 16
Özışık, Hatice
10, 15, 16, 18, 27
Öz, İlker
61, 63
Özkaya, Mesut
55, 60
Salim, Ozan
21
Samaras, Vassilis
34
Saraç Sivrikoz, Tuğba
47
Sarı, Ramazan
9, 21, 35
Saydam, Başak Özgen
14
Saygılı, Füsun
18, 27
Sayıner, Zeynel Abidin 55,
60, 65
Sezer, Havva
66
Soyluk Selçukbiricik, Özlem
22
Sökmensüer, Cenk
43, 46
Süleymanlar, Gültekin
35
P
Ş
Papatheodorou, Athanasios
34
Paschou, Stavroula A.
34
Peker, Önder
64
Peynirci, Hande
11
Polat, Şefika Burçak
32
Poyraz, Niüufer
48
Şarer Yürekli, Banu 10, 15, 16, 18,
27
Şimşek Bağır, Gülay
36, 38
Şişman, Pınar
11
Q
Qasim, Bayar Ahmed
31, 49
T
Tabur, Suzan
Tamer, Gonca
Tanakol, Refik
Tanrıkulu, Seher
Taşkıran, Bengür
Tatlıpınar, Arzu
Tazegül, Gökhan
Tekin, Sakin
20 - 23 October, 2016
70
55, 60
59
22
47
11, 44
19
21, 35
47
Topaloğlu, Oya
13, 48
Topçuoğlu, Elif
29
Tsiberkin, Alexandr
37
Tuncay, Mehmet
23
Tzioras, Konstantinos
34
U
Uygun, Burçin
45
Ü
Üçler, Rıfkı
Ünal, Mustafa
Ünlütürk, Uğur
67
61, 63
20, 26, 52
V
Vahabov, Javanshir
Vetem Karagülle, İnci
Vryonidou, Andromachi
56
44
34
Y
Yalçın, Mehmet Muhittin
23
Yaşar, Zeki
18
Yazıcı, Dilek
66
Yıldırım, Meltem
48
Yıldırım Şimşir, Ilgın
10, 27
Yıldız, Bülent Okan
25, 51, 56
Yılmaz, Nusret
9, 21, 35
Z
Zuhur, Sayid
Antalya - Turkey
40
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