Pelvic and Bladder Catecholamine-Producing Tumors A Review of the Literature

Abstract Background Although PGLs may arise at any site where physiologic paraganglionic tissue exists, pelvic origins are not frequent (about 2%), and the most common localization is the retroperitoneal space, whereas bladder PGLs account for less than 0.06% of all bladder neoplasms and less than 1% of all PGLs. Starting from the description of two interesting our cases, we wondered to review the literature on pelvic and bladder paragangliomas (PGLs) with the aim of illustrating clinical characteristics and current management, and enlightening the needed of a prompt diagnosis to avoid the onset of several complications.Methods We herein performed a brief review of the literature about pelvic PGLs in the PubMed library, conducted according to the PRISMA statement, including studies published from 1954 to 2019. We also discuss clinical presentation, morphologic appearance, and management of two cases of pelvic PGL, both of them characterized by a history of paroxysmal high blood pressure episodes uncontrolled by antihypertensive therapy associated with disabling symptoms, misdiagnosed for many years.Results Overall, we evaluated 108 case reports. The majority were females, presenting typical signs and symptoms, such as arterial hypertension (54.2%), hematuria (35.1%), and headache (33.6%). After treatment, the clinical and biochemical remission was showed in only 55% of analyzed cases.Conclusions For their singular localization, pelvic and bladder PGLs often might be misdiagnosed. The resulting delay in the proper treatment could be responsible for the relative low percentage of complete clinical and biochemical remission.