星細胞腫を合併したMaffucci症候群の1例

Maffucci's Syndrome Associated with Primary Brain Tumor: Report of a case

I．はじめに

　血管腫と軟骨腫の多発するMaffucci症候群は比較的稀であり，中胚葉性異形成症と考えられている^4）．本症候群では軟骨腫および血管腫の悪性化や他臓器の悪性腫瘍との合併が知られているが^1,7,14,23），原発性脳腫瘍との合併例は現在までにわずかに8例^{2-4,7,10,13,21,22）}の報告があるにすぎない．

　最近，本症候群に星細胞腫（右前頭葉，fibrillarytype）を合併した症例を経験したので，若干の文献的考察を加えて報告する．

A rare case of Maffucci's syndrome associated with astrocytoma at the right frontal lobe and intracranial chondroma is reported in detail with a review of litera-ture.

A 24-year-old male who had diagnosed as multiplechondroma at the age of 14 years old was admitted to the hospital with complaint of double vision. Neurological examination revealed no abnormality ex-cept for the left abducens palsy. Multiple sub-cutaneous blueish nodules in the left hand was carried for biopsy as cavernous hemangioma. Craniogram showed expansive radiolucent area in the parasellar region.