Succinate Dehydrogenase–Deficient Paraganglioma of the Prostate

Paragangliomas emerging in unusual locations may represent a diagnostic pitfall. We report clinical, genetic, imaging, and histopathologic features of succinate dehydrogenase–deficient paraganglioma arising within the prostate gland. This patient had multiple and recurrent paragangliomas since the age of 18 years. During the course of surveillance for a pathogenic germline SDHB variant, he developed 2 separate foci of disease in the prostate and retroperitoneum. Both lesions were gallium 68 DOTATATE positron emission tomography–avid and proved to be separate paragangliomas histologically. We emphasize that the prostate represents a site for paragangliomas, particularly in genetically predisposed patients who develop enlargement of the gland with normal prostatic-specific antigen.