The Usual Story of an Unusual Site: A Case Series of Urinary Bladder Paraganglioma from a Single Institution
Abstract
Objective: Bladder paragangliomas (PUB) are extremely rare. The incidence is <0.06% of all bladder tumors and <1% of all pheochromocytomas. The importance of accurate diagnosis is crucial for effective patient treatment.
Material and Methods: This study encompassed 9 cases of bladder paragangliomas, identified over a duration of 6 years. Analyses of the epidemiological features, symptoms, imaging, laboratory tests, treatments, pathology, immunohistochemistry (IHC), and follow-up outcomes were executed.
Results: Among the 9 cases of PUB, 5 were female and 4 male, with ages ranging from 24 to 73 years. The most common presenting symptom was painless gross hematuria (67%), followed by micturition attack (22%), and hypertension (11%). Radiologically, the tumors were well circumscribed, solitary, and broad-based. Most patients underwent transurethral resection of the bladder tumor (TURBT). The characteristic Zell Ballen pattern of tumor cells, separated by a delicate fibrovascular network and supported by small sustentacular cells, was evident on microscopy. All tumors were classified as T2, per the 8th edition American Joint Committee on Cancer Staging System (AJCC) staging system. Based on histmorphology, various differentials were considered. On IHC, tumor cells were immunoreactive for synaptophysin, chromogranin, with S100 highlighting sustentacular cells, thereby confirming the diagnosis of PUB. MIB1 ranged from 1-10%. Two patients were lost to follow-up. Duration of follow-up ranged from 3 months to 78 months (6.5 years). All the patients were disease and symptom-free at the follow-up.
Conclusion: PUB is a rare condition. Characteristic clinical presentation, histologic features, and application of immunohistochemistry are all important to differentiate this tumor from other bladder tumors, ensuring patients receive the appropriate treatment.
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