A case of prostatic rhabdomyosarcoma in an 8-year-old boy is presented. He was referred to Kurobe City Hospital with chief complaints of urinary retention and fever. Radiologic examinations revealed a huge prostatic tumor. Prostatic needle biopsy was performed and the pathological diagnosis was embryonal rhabdomyosarcoma of the prostate. He was referred to our hospital and treated with chemotherapy consisting of cisplatin, vincristine, cyclophosphamide, adriamycin, actinomycin-D and radiotherapy based on the regimen of IRS III. Total prostatectomy was performed 6 months after the start of therapy. Viable tumor cells were found in the prostate and the left obturator lymph nodes. After the operation, we continued chemotherapy. No recurrence was observed 8 months after the operation. However, local recurrence occurred in the pelvis 10 months after the operation and he died 2 months after the recurrence.

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