Introduction:
Approximately 10% of pheocromocytomas occur at extra-adrenal sites, with <0,05% accounting for bladder paragangliomas (PG).
These tumors are typically benign, and the most common symptoms are hypertension, headache, hematuria and palpitations, with characteristic micturition attacks.
Complete surgical resection of the tumour through Transurethral Ressection of Bladder Tumor (TURBT) or partial cystectomy can be curative in many cases.
Life-long clinical and biological follow-up of these patients is essential.
Objective, Material, Methods and Results:
History of a previously healthy 53 years old male patient with a history of 3 episodes of painless gross hematuria, and 1 micturition syncope.
A contrasted-enhanced CT scan (CECT) was performed and a highly suspected 67*78mm right kindey mass was detected. Plus, there was a contrast enhanced bladder mass with full wall invasion. No lymph nodes or metastases detected.
During the TURBT procedure he became severely hypertensive but controled under antihypertensive drugs.
Histological examination showed presence of a PG.
At 1-month postoperatively, plasma metanephrines (PM), and urinary vanillylmandelic acid (VMA), were still high and the CE-CT scan + cistoscopy suggested residual tumor.
The patient underwent radical nephroureterectomy + cystoprostatectomy. Histological examination showed a kidney clear cell carcinoma and bladder PG.
24h urine showed a dramatic drop in VMA level after the surgery until now. He is still well without recurrence.
Conclusion:
PG of the urinary bladder is very rare and painless hematuria is a common presenting complaint. Functional assessment of plasma and/or urine catecholamine levels are helpful in the initial workup and in the follow-up afterward.
Alpha-adrenergic blockade and preoperative volume expansion have been shown to significantly reduce peri-operative mortality and morbidity.
Many advocate complete surgical removal as the standard management for paragangliomas, including partial cystectomy or radical cystectomy, because it originates from the intra-mural portion of the bladder wall, and therefore it´s difficult to remove the tumor entirely with TURBT only.
Because bladder PG are likely to recur and to metastasize, lifelong follow-up with appropriate history, annual measurement of plasma and urinary catecholamine levels and cystoscopy is essential.