Extraadrenal pheochromocytomas, or paragangliomas, are rare and potentially malignant tumors that may occur in extraadrenal chromaffin tissue. The determination of malignant or benign represents the crucial point once the diagnosis has been established. We report a case of a 33-year-old man with an incidental paraganglioma involving the prostate. He underwent nerve-sparing radical retropubic prostatectomy and monolateral lymphadenectomy. Urinary continence was complete on postoperative day 5, and the patient had intercourse 2 weeks after surgery. The follow-up was at 24 months with no evidence of recurrent disease on clinical examination or body scan evaluation.
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