A Rare Case Of Functional Extra-Adrenal Urinary Bladder Paraganglioma In A 19-Year-Old Female Managed With Partial Cystectomy And Ureteral Reimplantion Using A Multidisciplinary Approach

Abstract

Objective: We report the multidisciplinary management of an unusual case of functional urinary bladder paraganglioma in a 19-year-old hypertensive female patient. Methods: We present the complete clinical presentation, imaging, operative details, histopathology, and follow-up of an unusual case of functional urinary bladder paraganglioma. Results: This case was unique because it presented as a functional tumor arising from urinary bladder in a young patient with uncontrolled hypertension. After biochemical, anatomical, and functional imaging, the diagnosis of a functional urinary bladder paraganglioma was confirmed. Adequate preoperative medical management was given to stabilize her blood pressure. Surgery was performed after proper optimization and explaining all of the high risk factors and expected complications of the procedure. Meticulous surgical planning was done to achieve complete tumor resection without any major complications or compromising the patient's fertility. Genetic analyses of related genes, including succinate dehydrogenase subunit B, were negative. Plasma normetanephrine levels of her first degree relatives were normal. She remained normotensive and asymptomatic at a 1.5-year follow-up. Conclusion: A multidisciplinary team approach consisting of an endocrinologist, a urologist, an anesthesiologist, and support staff is important for the successful management of high-risk cases of functional urinary bladder paraganglioma. (AACE Clinical Case Rep. 2017;3:e79-e82) Abbreviations: MIBG = metaiodobenzylguanidine; SDHB = succinate dehydrogenase subunit B e80 Urinary Bladder Paraganglioma, AACE Clinical Case Rep. 2017;3(No. 1)