Malignant paraganglioma of the urinary bladder in a 10 year old

Posted By Lena Naffaa
Malignant paraganglioma of the urinary bladder in a 10 year old

INTRO: Pheochromocytomas are tumors of chromaffin cells that arise from the adrenal gland. They can, however arise in 10-20% of the cases from sympathetic tissue outside the adrenals and are then called paragangliomas. Paragangliomas of the bladder are very rare, representing 0.06% of bladder tumors. These are typically functional and present most commonly with symptoms of paroxysmal hypertension, palpitations, and micturition syncope while a large proportion of cases presenting with nonspecific symptoms of a bladder tumor such as painless hematuria, abdominal fullness and pain. CASE: A 10-year-old boy presented with to his primary care physician with a 4 month- history of a weak urinary stream, occasional headache, sweating and dizziness when straining for defecation or urination. Physical examination and vital signs on presentation were unremarkable, in particular, the heart rate and blood pressure were within normal limits. Urinalysis on presentation was negative for hematuria but showed trace protein. Ultrasound of the pelvis was done and showed a 3 cm echogenic bladder mass. Further characterization by MRI of the pelvis showed a 2.5 x 2.7x 2.4 cm (AP, craniocaudal and transverse diameters) lobulated mass centered at left posterior wall/bladder base protruding into the bladder lumen. Following contrast administration, the mass showed significant enhancement with a non-enhancing center suggestive of necrosis. On diffusion, mild restriction was seen at its peripheral aspect. He then underwent a CT guided biopsy of the mass with no complications. Immunohistochemistry of the tumor cells was positive for synaptophysin, chromogranin, CD56 and INI-1. It was negative for Myogenin, Desmin, CKAE1/3, CD99, WT-1, S-100 and Melan A. Ki-67 was low. The favored diagnosis was paraganglioma. IMAGE LEGEND: MRI of the pelvis: Coronal (A) and axial (B) T2WI with fat suppression demonstrate a hypointense mass. (C) T1WI image shows an intermediate to slightly hypointense mass arising from the left posterior wall of the bladder (D) Shows significant enhancement post gadolinium administration. (E) The enhancement is also evident on fat suppressed T1WI post contrast (SPIR). (F) T2WI the mass is showing hyperintense T2 signal, typical of pheochromocytoma/paraganglioma. (G) Axial DWI: the mass is showing restricted diffusion with a high signal on diffusion images and (H) low signal on the ADC map

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