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神經纖維瘤病併發頸部神經纖維肉瘤-病例報告

Neurofibromatosis Associated with Neurofibrosarcoma - Case Report

摘要


神經纖維肉瘤(neurofibrosarcoma),或稱梭狀細胞肉瘤(spindle cell sarcoma),是罕見的惡性腫瘤,佔所有軟組織肉瘤的5%;它可與神經纖維瘤病(neurofibromatiosis or von Recklinghausen’s disease)合併發生,估計約3-30%,但也可獨立發生。本科於民國82年8月經歷1例合併頸部梭狀細胞肉瘤的神經纖維瘤之病例,經手術做大範圍切除後,並施予術後放射治療,追蹤一年迄今,並無復發跡象,謹提出此報告。

並列摘要


Neurofibrosarcoma is a rare malignant tumor which comprises about 5% of soft tissue sarcoma. The neoplasm may arise independently of or in association with neurofibromatosis (von Recklinghausen’s disease). The incidence of neurofibrosarcoma arising in neurofibromatosis has been estimated to be 3-30% according to various reports. We experienced a case of neck neurofibromatosis in August 1993. The patient underwent a wide surgical excision combined with postoperative radiotherapy, and there is no evidence of recurrence after one year follow-up.

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