Primary malignant peripheral nerve sheath tumor of prostate in a young adult: A case report.

RATIONALE

Prostate 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 CONCERNS

A 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.

DIAGNOSES

Core 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.

INTERVENTIONS

Adjuvant 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.

OUTCOMES

The best response to chemotherapy was partial response. After several courses of chemotherapy, he died 9 months after the surgery.

LESSONS

Primary 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.