[Neurofibrosarcoma of the perineum associated with von Recklinghausen's disease: a case report and review of the literature].
A case of perineal neurofibrosarcoma associated with von Recklinghausen's disease is reported. The patient was a 30-year-old man, who complained of a mass between the scrotum and anus. The mass was asymptomatic. On Jan. 13, 1981, simple excision was performed. Histological examination revealed neurofibrosarcoma. After 3 months he had recurrence of the perineal tumor. Combined chemotherapy and radiation therapy were performed. But, his condition became worse due to general metastasis and he died 12 months after operation. A total of 22 cases of perineal neoplasms are reviewed.
D000328:Adult; D003131:Combined Modality Therapy; D005834:Genital Neoplasms, Male / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D009455:Neurofibroma / Q000473:pathology* / Q000628:therapy; D009456:Neurofibromatosis 1 / Q000473:pathology*; D010502:Perineum; D012878:Skin Neoplasms / Q000473:pathology*