Abstract :
Background: Extra-adrenal chromaffin related tumors are called Paraganglioma. These tumors constitute
18% of extra-adrenal pheochromocytomas and are exceptionally rare in the bladder accounting for of bladder tumors.
Case report: A 45-year-old female came with history of painless hematuria of 3 days duration. On MRI
scan, she was found to have a bladder tumor arising from the posterior wall. Transurethral resection of
the bladder tumor (TUR-BT) was done. During the procedure, the patient had a sudden increase in blood
pressure with acute left ventricular failure and pulmonary edema. Multiple grey brown TUR-BT specimen
bits on microscopy showed tumor cells in “Zellballen” pattern. These cells were round to polygonal
with abundant granular cytoplasm, round, regular nuclei with stippled chromatin. Mitotic figures were
seen occasionally. Immunohistochemistry for Chromogranin A showed strong positivity confirming the
diagnosis to be paraganglioma of the bladder. Follow-up cystoscopy of the patient didn’t show any evidence
of the tumor, also her urinary and plasma vanillylmandelic acid (VMA) levels were within normal range
post-operatively.
Conclusion: Paraganglioma of the bladder is extremely rare. Our case presenting with only painless gross
hematuria was a close mimicker of urothelial carcinoma. It has a benign coarse but has a very high
recurrence rate. It is essential to differentiate it from its histological differentials for proper management
and follow-up.
Keyword :
Chromaffin tumors, Extraadrenal Pheochromocytoma, Paraganglioma, Bladder tumor, Painless hematuria, Chromogranin A.