von Recklinghausen病に合併した頭頸部malignant schwannomaの1例

Malignant Schwannoma of the Head and Neck associated with von Recklinghausen Disease: Report of a Case

I．はじめに

　schwannomaは代表的な良性腫瘍の一つであるが時に悪性転化することも知られており，その頻度は2.0〜15％^1〜3）といわれている。一方malig—nant schwannoma （以下MSと略記）はその約10％が頭頸部に発生^4〜8）し，さらにvon Reckling—hausen病（以下R病と略記）に合併することが多い^9,10）とされる。今回われわれはR病の患者で幼少時より頭頸部腫瘤を有し，約8年を経過した後，悪性転化し急速に増大したMSの1剖検例を経験したので，若干の文献的考察を加えて報告する。

A 16-year-old female had cafe-au-lait spots of skin at birth. At the age of 8 years, a soft sub-cutaneous tumor was noticed in the occipital region. The tumor had begun to grow at 16 years of age. Radiologically the occipital bone and the arch of atlas were eroded by the tumor. Removal of the tumor was performed subtotally. The tumor was found as malignant schwannoma microscopically. The residual tumor grew up im-mediately, and surgical treatments with irradia-tion and chemotherapy were performed at 4 different occasions. After pulmonary metastases were recognized, she died of cachexia about 10 months after the admission.