Int J Cancer Manag. 2019 January; 12(1):e86352. Published online 2019 January 8. doi: 10.5812/ijcm.86352. Case Report Painless Gross Hematuria: A New Presentation of Primitive Neuroectodermal Tumor of the Prostate Babak Javanmard 1 , Morteza Fallah Karkan 1, * , Mohammad Reza Yousefi 1 and Mahsa Ahadi 2 1 Department of Urology, Shohada-e-Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran 2 Department of Pathology, Shohada-e-Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran * Corresponding author: Resident of Urology, Laser Application in Medical Sciences Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Tel: +98-2122736386, Email: mortezafallah.md@gmail.com Received 2018 November 12; Revised 2018 December 01; Accepted 2018 December 30. Abstract 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 re- section 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 prog- nosis is very poor, medical staff should pay enough attention to the differential diagnosis, choosing the best treatment and subjects close follow-up. Keywords: Primitive Neuroectodermal Tumor, Extra-Skeletal Ewing’s Sarcoma, Prostate Sarcoma, CD99 1. Introduction Gross hematuria has a wide differential diagnosis (1- 4). Primary prostate sarcoma, which derives from the mes- enchymal cells of the prostate stroma and is responsible for less than 0.1% of primary prostate cancers, was first re- ported in 2003. Primitive neuroectodermal tumor (PNET) or extra-skeletal Ewing’s sarcoma is a very rare type of prostate sarcoma that mostly occurs in young adults, and is associated with a poor prognosis (5-7). PNET is classified to central and peripheral types according to their locations (3). Cluster designation (CD) 99 positivity distinguishes PNET from other entities such as rhabdomyosarcoma, lym- phoma, and neuroendocrine cancer (8). 2. Case Presentation A 37-year-old male was admitted to emergency depart- ment with intermittent painless gross hematuria from 1 month prior to admission. The medical and familial his- tories were unremarkable. He had no documentation of previous malignancies. Digital rectal examination demon- strated a very large prostate with poor mobility. There were no palpable lymph nodes. Ultrasonographic eval- uations revealed normal kidney structure, bilateral hy- dronephrosis, distended Bladder filled with clot, and a huge prostate. According to orthostatic blood pressure changes and hemoglobin of 7mg/dL, conservative therapy initiated and patient was transferred to operation room (OR) urgently. Cystoscopy and bladder washing was done and bleeding origin was detected from prostate lodge. The day after that, the patient was reevaluated in OR because of the persistent bleeding. Trans-urethral resec- tion of prostate (TURP) (3 bites for tissue sampling), ful- guration, bilateral ureteroscopy, and rectoscopy were per- formed and, finally, TRUS Bx of prostate was done. The pa- tient’s serum prostate-specific antigen (PSA) level was 1.07 ng/mL, and all of the other serum tumor markers (such as carbohydrate antigen 19 - 9, carcino-embryonic antigen, alpha-fetoprotein, and lactate dehydrogenase) were nor- mal. Histological findings were consistent with severely in- flamed prostatic urethra, papillary heperplasia, and reac- tive atypia with no evidence of malignancy. The PSA and immunohistochemistry (IHC) results of alpha-methylacyl- Copyright © 2019, Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.