• Hyojeong Kim, KimDo YoungDYDepartment of Hematology-Oncology., Young Mi Seol, Ja Yoon Ku, Kyung Un Choi, and Young Jin Choi.
• Department of Hematology-Oncology.
• Medicine (Baltimore). 2018 Sep 1; 97 (39): e12040.

RationaleProstate sarcoma has been reported to represent 0.7% of primary prostate malignancies. Leiomyosarcoma and rhabdomyosarcoma are the most common sarcomas of the prostate. Malignant peripheral nerve sheath tumor (MPNST) of the prostate is very rare.Patient ConcernsA 22-year-old man presented with gross hematuria and voiding difficulty for 2 weeks. Magnetic resonance imaging showed a 6-cm mass in the left lobe of the prostate.DiagnosesCore needle biopsy results revealed high-grade sarcoma, suggestive of poorly differentiated synovial sarcoma. The final diagnosis of laparoscopic prostatectomy was MPNST, because it did not show the presence of SYT-SSX fusion transcripts on reverse transcription polymerase chain reaction analysis.InterventionsAdjuvant radiotherapy was planned because preoperative positron emission tomography-computed tomography (CT) did not show any metastatic lesion and the resection margin was microscopically involved. However, chest CT showed multiple lung metastases a month after prostatectomy. A chemotherapeutic regimen of doxorubicin and ifosfamide was administered.OutcomesThe best response to chemotherapy was partial response. After several courses of chemotherapy, he died 9 months after the surgery.LessonsPrimary prostate sarcoma and even MPNST are extremely rare. MPNST of the prostate has seldom been reported. This report may help diagnose and manage the disease.

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