Endocrine Abstracts

Introduction: Paraganglioma is a rare neuroendocrine neoplasm which tends to develop in the extra-adrenal glands. Paraganglioma in the bladder is extremely rare and accounts for 0.06% of all bladder tumors Herein we report a case of paraganglioma that was mistaken for urothelial carcinoma and revealed by an intra-operative hypertensive crisis with bradycardia.

Case presentation: A 48-year-old woman was referred to the urology department for intermittent terminal hematuria. The medical history included primary hypothyroidism and uterine Fibroids. On examination the patient appeared well built, normotensive with a heart rate of 78 bpm. Orthostatic change in blood pressure was not documented. Urinalysis revealed full field RBC and urine culture was negative. Ultrasound and Computerized tomography urogram revealed a 2.5-cm solid mass confined to the posterior wall of the bladder and an additional focal liver lesion that the dynamic enhancement pattern recall a hemangioma Decision was made to go for transurethral resection of tumor. Early during the resection, her blood pressure started to raise up to 220/110 mmhg and her heart rate decreased to 35 bpm So the procedure was held. Post operatively, her blood pressure was observed for 24 hours and showed tendency toward hypotension. Her heart rythm was monitored for 24 hours and it was normal. The pathological picture of the resected biopsy confirmed the diagnosis of Paraganglioma of urinary bladder. The 24-hour urine study revealed elevated levels of norepinephrine (0.5 nmol/l), epinephrine (0.16 nmol/l). MIBG scintigraphy detected a mass confined to the bladder without any obvious multicentricity. Six months later, the patient underwent radical removal of the tumor after being treated with a 10 day course of α and B blockade and adequate hydration. Post surgical MIBG scan didn’t detect any functional lesion. In twenty-five months follow-up, patient’s blood pressure, urinary VMA and epinephrine remained normal.

Conclusion: Urinary bladder paraganglioma is an extremely rare entity. The classical triad of episodic hypertension, hematuria and post-micturition syncope is virtually diagnostic, but is very rare. Our patient presented with hematuria but no hypertension. The correct preoperative identification of paraganglioma is important. However, it is often overlooked because of its rareness. Unsuspected ones may result in intraoperative hypertensive crises, as in our case, and greatly increase the perioperative mortality forcing the surgeon to terminate cystoscopic tumor resection. This case highlights variable presentation of paraganglioma, importance of having a high index of clinical suspicion for early recognition and prompt management.