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24 yo man with hypertension
Endocrine Post Clinic Conference
September 2, 2011
Outline: Pheochromocytoma
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Case presentation
CT results
Imaging algorithms
Role of MIBG
Other diagnostic tools
Genetics
Case discussion
Case : H&P
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Asymptomatic SBP 230s in 2009 on routine postdeployment exam
Reports elevated catecholamines but no findings on
CT / MR / MIBG
Tried on beta-blockers which made him feel lousy. +
orthostasis
Now with sweats, palpitations, panic attacks, occasional
left sided chest pain. +history of migraines, unchanged
Currently without treatment, has tried to avoid physical
stresses
Case 1: H&P
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Past Medical History:
• Hypertension first noted 2009
• Migraines since age 12
• PTSD
• no prior surgeries
Case 1: H&P
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Medications:
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Social:
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xanax prn anxiety
smokes <1 ppd
occ EtOH
no cocaine/meth or other illicits
Family:
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Immediate family healthy without hypertension
Case 1: H&P
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Physical Exam:
T 99.3, HR 86, 180/132, 100% on room air
Well-developed, well-nourished male, NAD
Eyes: PERRL, EOMI, no lid lag, no stare, vision
full to confrontation
Neck: no thyromegaly, no masses, no nodules
RRR without m/r/g
CTAB
Abd benign
Skin warm and mildly diaphoretic
Livedo reticularis?
Livedo reticularis?
Case: Laboratory Data
139
104
19
3.7
28
1.0
127
9
16
48
220
(plt giant and clumped)
Calcium 9.4
Calcitonin < 0.2
LFTs normal
Albumin:
4.4
Thyroid function tests
normal
Urine
normeta 9125 (<600)
metaneph 9256 (<900)
Plasma
normeta 4258 (<149)
metaneph 4302 (<206)
catechol 12182 (<504)
“Heterogeneously enhancing, partially necrotic right
adrenal mass measuring 3.8 cm . . .”
CT
Next step?
Ilias I , Pacak K JCEM 2004;89:479-491
MIBG
Controversy: All cases or only when unable
to find a typical tumor or when high
suspicion for malignancy?
Malignancy associated with extra-adrenal
location, larger tumor size and +SDHB
mutation
Metastatic tumors larger, present at younger
age and more likely necrotic on path
Metastatic pheochromocytoma: Does the size and age matter?. European Journal of Clinical
Investigation. doi: 10.1111/j.1365-2362.2011.02518.x
MIBG
Recent meta-analysis placed sens/spec at
94% and 92% for pheo
In a study of 32 patients, able to reach
100% sensitivity and ppv with
MRI+MIBG. Only false negatives
(three) with MIBG alone were either
small or necrotic intraadrenal masses
The Journal of Clinical Endocrinology & Metabolism June 1, 2010 vol. 95 no. 6 2596-2606
Nuclear Medicine Communications July 2006 vol. 27 no. 7 583-587
Patient’s MIBG
Right
Left
Inverted MIBG image
Other diagnostic tools
PET > MIBG?
A 2009 prospective observational study of 52 patients
found greater sensitivity for metastatic disease with
PET/CT than MIBG
The Journal of Clinical Endocrinology & Metabolism December 1, 2009 vol. 94 no. 12 4757-4767
Other diagnostic tools
Adrenal vein sampling
High variability even in normal patients increases
risk of false positives
Adrenal venous sampling for catecholamines: a normal value study. Clin Endocrinol Metab.
2010;95(3):1328
Syndromes
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MEN 2
vonHippel Landau
Neurofibromatosis
Familial paragangliomas
When to test genetics?
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1Clin
1/3 pheos carry germline mutation
In one study, of 989 apparently nonsyndromic patients, 342 with mutation.
Only 8 of these missed if ignored
age>45, only single adrenal pheos and
those without prior head/neck
paraganglionomas
Cancer Res October 15, 2009 15; 6378
Plan for this patient
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Continue alpha blockade in prep for OR
MRI to better visualize left adrenal
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If no clear mass in left adrenal, adrenal
venous sampling? Just leave left side
alone? Take out whole left adrenal?
Consider genetic testing