Functional Bladder Paraganglioma as an Incidental Finding During Infertility Workup

Abstract

Bladder paragangliomas (PGLs) are extremely rare catecholamine-producing neuroendocrine tumors. They arise more frequently in the trigone and have unpredictable depth and behavior. Most cases typically present with a well-defined set of symptoms triggered by micturition or bladder overdistension. Besides long-term follow-up, they are usually managed by either transurethral resection (TUR) or partial cystectomy (PC). However, about 25% of all documented cases do not manifest clinically, raising both diagnosis and management challenges. This report describes an unusual case of a misdiagnosed, functional PGL arising on the bladder dome, which was fortuitously detected in a 21-year-old female during a fertility workup. Owing to its hypervascular nature and submucosal location, bladder PGL was suspected on ultrasound and CT findings and successfully diagnosed before surgery through biochemical confirmation. It was managed by cystoscopy-guided laparoscopic partial cystectomy (LPC) with good oncological and urinary outcomes, as well as no compromise of her fertility potential. To our best knowledge, this is the first case to be incidentally detected on transvaginal ultrasound during evaluation for infertility. This case also stresses the importance of considering PGL in the differential diagnosis of atypical bladder tumors, as well as conservative management through simultaneous laparoscopy and cystoscopy, when approaching young patients with large functional PGL.

A retrospective study of paraganglioma of the urinary bladder and literature review

“…Less than 10% paraganglioma are in the urinary bladder, and the young people are more easily to develop PUB. PUB arise more frequently in the trigone of bladder with an average size of 3.9cm [5]. To the best of our knowledge, we have reported the largest number of PUB patients in one center until now.…”

Bladder paraganglioma – Case report on a rare but important differential

“…These are very rare tumours, accounting for <0.05% of bladder tumours. 1 A bladder paraganglioma is even more rare, consisting <1% of all paragangliomas. 1 Presentation is variable, given that only 1–3% secrete enough catecholamines to be clinically significant, but can cause unexplained hypertension, headaches, palpitations, sweating and hot flushes, as well as micturition or bladder distension syncope.…”

“… 1 A bladder paraganglioma is even more rare, consisting <1% of all paragangliomas. 1 Presentation is variable, given that only 1–3% secrete enough catecholamines to be clinically significant, but can cause unexplained hypertension, headaches, palpitations, sweating and hot flushes, as well as micturition or bladder distension syncope. 1 Paragangliomas can mimic other bladder tumours during investigation for haematuria or lower urinary tract symptoms and cause issues during surgical resection if not identified pre-operatively.…”

“… 1 Presentation is variable, given that only 1–3% secrete enough catecholamines to be clinically significant, but can cause unexplained hypertension, headaches, palpitations, sweating and hot flushes, as well as micturition or bladder distension syncope. 1 Paragangliomas can mimic other bladder tumours during investigation for haematuria or lower urinary tract symptoms and cause issues during surgical resection if not identified pre-operatively. The following case describes an incidental bladder paraganglioma discovered during routine flexible cystoscopy in a young female with non-visible haematuria.…”