Introduction: Primitive neuroectodermal tumor (PNET) is a very rare type of prostate sarcoma that mostly occurs in young adults, and it is associated with a poor prognosis. Case Presentation: A 37-year-old male was admitted with intermittent painless gross hematuria from 1 month prior to admission. Cystosocopy, abdominopelvic computed tomography scan (ACTS), and magnetic resonance imaging (MRI) revealed huge prostate. All tumor markers were negative and pathology findings of trans-rectal ultrasonographic biopsy (TRUS Bx) and trans-urethral resection of prostate (TURP) were consistent with severely inflamed prostatic urethra with no evidence of malignancy. The patient underwent radical prostatectomy. Histopathology of the specimens showed malignant neoplasm of small round and oval cells suggestive of PNET. Immunohistochemistry (IHC) study results on CD99 were positive. Conclusions: We report a rare uncommon case of prostate PNET presented by intermittent painless gross hematuria. As the prognosis is very poor, medical staff should pay enough attention to the differential diagnosis, choosing the best treatment and subjects close follow-up.
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