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A 20-year-old Male with Back Pain
Surabhi Batra, MD; Anita Gupta, MD; and Radhika Peddinti, MD
A
20-year-old, previously
healthy, white male, presented with low back and
right flank pain for 2 weeks. The
pain was varying in intensity from
moderate to severe and diffuse and
radiating to the groin, with no exacerbating or relieving factors and
minimal relief with ibuprofen. He
said the pain started after he was in
a high velocity motor vehicle accident. According to the patient, his
car was hit from behind by another
vehicle, which resulted in skid-
Surabhi Batra, MD; and Anita Gupta,
MD; are pediatric residents at John H.
Stroger Jr. Hospital of Cook County, Chicago. Radhika Peddinti, MD, is attending
physician, John H. Stroger Jr. Hospital of
Cook County.
Address correspondence to: Surabhi
Batra, MD, 903 S. Ashland Ave. Apt-1114B,
Chicago, IL 60302; fax: 312-864-9717; or
e-mail: [email protected].
Dr. Batra; Dr. Gupta; and Dr. Peddinti
have disclosed no relevant financial relationships.
doi: 10.3928/00904481-20100922-03
ding and the patient’s car landing
upside down. There was no loss
of consciousness. The patient was
evaluated by the emergency medical system after the accident and
was discharged after a computer-
On physical examination, he
was a well-appearing male
with a blood pressure of
172/94, heart rate of 92 bpm,
respiratory rate of 14 bpm,
and temperature of 100.8° F.
ized tomography (CT) scan of the
head failed to show any evidence
of injury or bleed. The laboratory
investigations done at that time revealed low hemoglobin.
Further questioning revealed
that the patient had been constipated for 1 month and had multiple episodes of flushing, usually
coinciding with the increase in
pain severity. His past history was
significant for bipolar disorder,
depression, and attention-deficit/
hyperactivity disorder (ADHD).
He had been taking divalproex sodium for 3 months. He admitted
non-compliance with medication.
He had been hospitalized numerous times for psychiatric issues.
The last hospitalization was 1 year
ago for suicidal ideation.
On physical examination, he was
a well-appearing male with blood
pressure of 172/94, heart rate of 92
bpm, respiratory rate of 14 bpm, and
temperature of 100.8°F. There was
tenderness and slight fullness in the
right lower quadrant. There were a
few 1- to 2-cm, enlarged anterior
cervical lymph nodes. The patient
had small, itchy, painful, red nodules, up to 1 cm, which were widely
spread over his face, neck, back,
and abdomen, with some degree of
mucositis. The rest of the physical
exam, including the neurological
exam, was non-contributory.
Over the next few days, the patient had a wide variability of blood
For diagnosis, see page 611.
Editor’s note: Each month, this department features a discussion of an unusual diagnosis in genetics, radiology,
or dermatology. A description and images are presented, followed by the diagnosis and an explanation of how the
diagnosis was determined. As always, your comments are welcome via e-mail at [email protected].
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case challenges
pressure, ranging from 190/94 mm
Hg to 134/60 mm Hg. The laboratory investigations performed at that
time were significant for hemoglobin of 9.5 g/dL; a total white count
of 5.95 k/uL with 63% neutrophils;
26% lymphocytes; and a platelet
count of 329 k/uL. The blood cultures drawn when the patient was
febrile were negative. Liver function tests were done and were within normal range. Serum phosphorus
was slightly elevated at 5.6 mg/dL.
A CT scan of the abdomen revealed
a large, right adrenal mass with a
large calcified component and multiple scattered lucencies throughout
the spine and pelvis.
As conventional treatment for
pain control failed to show any
improvement, the patient was admitted and started on a hydromorphone PCA pump. Magnetic resonance imaging (MRI) of the spine
revealed diffuse bone marrow
irregularities, with moderate to
severe right neural foraminal narrowing at the level of T9-10 and
T11-12. A CT-guided biopsy of the
right adrenal mass and a bone marrow biopsy were performed, which
revealed the diagnosis.
DIAGNOSIS
Neuroblastoma
The CT-guided biopsy from the
right adrenal mass was reported as
neuroblastoma with poor stroma,
some differentiation, low mitotic
karyorrhexis index (MKI), and an
unfavorable histology. Following
this, a bone marrow biopsy was
performed, which revealed diffuse
involvement of the bone marrow
with small, round blue cells.
DISCUSSION
Although neuroblastoma is the
most common solid extra-cranial
neoplasm of children, it is rarely
reported in adults, with fewer
than 10% of cases occurring after
10 years.1
Neuroblastoma is a tumor arising from the embryonal cells of the
sympathetic nervous system. The
signs and symptoms of the disease
may vary, depending on the site of
the primary tumor. Bone pain due
to metastasis to the bones and the
marrow is a common presentation.
Sometimes, patients have neurological deficits because of the compression of the spinal cord by bony
metastasis. Some patients may also
present with generalized malaise,
fatigue, fever, and other constitutional symptoms.
Some specific symptoms associated with neuroblastoma, although
not exhibited by our patient, are
periorbital ecchymosis, also known
as raccoon eyes, due to infiltration
of the periorbital tissue by the tumor cells, and intractable diarrhea,
due to secretion of vasoactive intestinal peptide by the tumor cells.
This is more common in younger age groups; nearly two-thirds of
patients with neuroblastoma have
abdominal primaries.2 Approximately 2% of patients present with
opsoclonus myoclonus, a paraneoplastic syndrome characterized by
Figure 1. CT scan of the abdomen revealed a large, right adrenal mass with a large calcified component and multiple scattered lucencies
throughout the spine and pelvis.
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TABLE.
Comparisons among Neuroblastoma and Pheochromocytoma and Ganglioneuroma
Characteristic
Neuroblastoma
Pheochromocytoma
Ganglioneuroma
Age at diagnosis
< 10 years mostly1
30-40 years2
Mean 7 years
Presentation
Abdominal mass2
Paroxysmal hypertension2
Mass effect, 50% asymptomatic 8-10
Most common primary site
65% abdomen, chest, pelvis,
Adrenal medulla 50%2
Posterior mediastinum 38%,
5-7
retroperitoneum8-10
and neck
Lymphatics, lungs, liver, bone2
Lymphatics
50%7
10%2
< 1%
92% increase in HVA and
Metanephrines more specific4
20% to 39%2
Most common site of metastasis
Lymph nodes, bone, and bone
Percentage that metastasizes
Urinary HVA, VMA,
metanephrines
marrow
2
VMA
the presence of myoclonic jerks and
dancing eyes. In infants, neuroblastoma may manifest as bluish lumps
under the skin, also known as blueberry muffin syndrome. About 92%
of the cases have hypertension as
the only presenting symptom.2
Most cases are diagnosed by biopsy from the primary site. Other
tests, including urine and plasma
homovanillic acid (HVA), vanillylmandelic acid (VMA), and Nmyc
gene amplification tests, can be
performed to support the diagnosis.
Although HVA is a metabolite of
dopamine, VMA is a metabolite of
norepinephrine. Their levels are increased in all tumors, which secrete
catecholamines. Most review of the
literature supports that it is rare for
adults with neuroblastoma to exhibit
increased excretion of HVA and
VMA or the Nmyc amplification.3
Our patient presented with
acute posttraumatic back pain,
with no constitutive signs or symptoms. His occupational history and
the severity of the motor vehicular
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2
crash were highly suggestive of a
traumatic etiology. Unresponsiveness of the pain to routine pain
medications made muscle sprain
less likely. Because there were no
neurological deficits, spinal cord
injury was disregarded. After the
physical exam revealed fullness in
the right lower quadrant, CT scan
of the abdomen was performed.
The findings of the CT suggested
bony metastasis with an adrenal tumor. At this, we broadened our differentials to include neuroblastoma,
malignant pheochromocytoma, paraganglioma, lymphoma, leukemia,
and teratoma. Considering that the
adrenal gland is the fourth most common site of metastasis, it was also
possible that the adrenal mass and the
bone lucencies were metastasis from
a distant primary tumor, such as from
the lungs, skin, thyroid, and colon.
Pheochromocytoma, neuroblastoma, and ganglioneuroma were
high on our differentials (see Table). Besides the adrenal mass and
bony metastasis, a high variability
in blood pressure and paroxysmal
episodes of flushing supported our
diagnosis.
In our patient, urine HVA and
VMA levels were done, which
showed very high levels of 31.6 mg/g
creatinine (normal range 1.4 to 5.3)
and 83.1 mg/g creatinine (normal
range 1.1 to 4.1), respectively.
According to the pathology report
of the adrenal mass biopsy and the
bone marrow findings, our patient had
International Neuroblastoma Staging
System (INSS) stage 4 neuroblastoma. Because of the rarity of the
cases occurring in the adult population, there is no standardized therapy
for adults, and most adults are treated
based upon the pediatric chemotherapy protocol. Myeloablative therapy
has been reported to be beneficial for
children with neuroblastoma in their
first remission; however, its utility in
older patients with neuroblastoma
has not yet been established.
He received cycle 1 of chemotherapy with topetecan, vincristine,
and cyclophosphamide, which he
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tolerated well. The skin lesions disappeared after treatment with topical bacitracin. Despite a low MKI,
this patient had a poor prognosis
because of his age at presentation
and because the disease was in stage
4 with poor differentiating stroma.
The patient was lost to follow-up.
CONCLUSION
Although rare in children older than 10 years, neuroblastoma
should be considered as the differential diagnosis of any patient presenting with an abdominal mass or
bone metastasis. Appropriate diagnosis should be established using a
biopsy from the tumor site.
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REFERENCES
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Marcus RB, Johnstone PA. Neuroblastoma in adults: incidence and survival
analysis based on SEER data. Pediatr
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JW, Pacak K. Emergencies caused by
pheochromocytoma, neuroblastoma, or
ganglioneuroma. Endocrinol Metab Clin
North Am. 2006;35(4):699-724.
3. Kushner BH, O’Reilly RJ, LaQuaglia M,
et al. Dose intensive use of cyclophosphamide in ablation of neuroblastoma.
Cancer. 1990;66(6):1095-1100.
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Connell JM. Comparison of diagnostic accuracy of urinary free metanephrines, vanillyl mandelic acid,
and catecholamines and plasma catecholamines for diagnosis of pheochromocytoma. J Endocrinol Metab.
2007;92(12);4602-4608.
5. Brossard J, Burnstein ML, Lemieux B.
Neuroblastoma: an enigmatic disease.
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6. Castleberry RP. Biology and treatment
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Current aspects of biology, risk assessment and treatment of neuroblastoma. Semin Surg Oncol.
1999;16(2):91-104.
8. Stowens D. Neuroblastoma and related tumors. AMA Arch Pathol.
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9. Radin R, David CL, Goldfarb H, et
al. Adrenal and extra-adrenal retroperitoneal ganglioneuroma: imaging findings in 13 adults. Radiology.
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10. Carpenter WB, Kernohan JW. Retroperitoneal ganglioneuromas and
neurofibromas: a clinicopathological
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