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

Abstract

Purpose . Starting from the description of two interesting cases, we wondered to review the literature on pelvic and bladder paragangliomas (PGLs) in order to illustrate clinical characteristics and current management, lightening the needed of a prompt diagnosis to avoid the onset of several complications. Although PGLs may arise at any site where physiologically chromaffin tissue exists, pelvic origins are not frequent (less than 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. 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 an history of paroxysmal high blood pressure episodes uncontrolled by antihypertensive therapy associated with disabling symptoms, not clearly understood 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 evident in only 55% of analyzed cases. Conclusions . For their singular localization, pelvic and bladder PGLs often might be misdiagnosed. The significant delay in the proper treatment could be responsible for the relative low percentage of complete clinical and biochemical remission.