Primary Paraganglioma of the Prostate: A Systematic Review of the Literature for A Rare Entity

Background Paragangliomas of the urinary tract are exceptionally uncommon, and sporadic case reports of primary paraganglioma of the prostate have been reported in the literature.Methods Systematic research in PubMed/Medline and Scopus databases concerning primary prostatic paraganglioma was performed by two independent investigators.Results This analysis included 25 adult males, with a mean age of 49.8 +/- 22.4 years. 32% of included patients had a history of hypertension. Problems during urination (52%), blood loss (44%), either as hematuria or hemospermia, and catecholamine-related symptoms (36%) comprised the most frequently reported clinical manifestations. Digital rectal examination found a palpable nodule in 36% of patients, while prostatic specific antigen (PSA) was normal in all tested patients. Abdominal ultrasound (44%), computed tomography (44%) and magnetic resonance imaging (28%) helped to identify the primary lesion. 24-hour urine epinephrine, norepinephrine and vanillylmandelic acid (VMA) levels were elevated in 90%, 80% and 90% of included patients. Open surgical excision of the mass was performed in 40%, transurethral resection in 8%, open radical prostatectomy in 24%, transurethral resection of the prostate in 16% and robot-assisted radical prostatectomy in 4% of included patients.Conclusion Due to atypical clinical manifestation and scarcity of prostatic paraganglioma, urologists should be aware of this extremely rare entity.