Choroid plexus carcinomaの1例

A case report of choroid plexus carcinoma

I．はじめに

　Choroid plexus tumor（以下CPT）は，小児脳腫瘍でも稀で，全脳腫瘍中1-3％を占める^10）．その中でもchoroid plexus carcinoma（以下CPC）はさらに稀であり，わが国では1969-1987年の間に14歳以下でわずか10例（全脳腫瘍中0.2％）が報告されているにすぎない^7）．CPCの主な好発部位は，側脳室に50％，第4脳室に37％，第3脳室に9％であり^6），7割が2歳迄に発症する^3）．また，この腫瘍は非常に易出血性で，全摘出が困難であること，くも膜下播種を起こすことなどから，平均生存率が9カ月と，これ迄，比較的治療困難な疾患であるとされてきた^3）．しかし，最近では，化学療法や放射線療法，あるいは積極的な摘出術の結果，平均生存期間が延び，機能的予後も良いという報告が散見される^2,4,5,8）．

　最近，われわれが経験した1歳の女児のCPCの症例を，若干の文献的考察を加え報告する．

Choroid plexus carcinoma (CPC) is a rare tumor with poor prognosis, whose optimal treatment has not yet been established. We report a case of a one-year-old infant who has been treated with successive combined therapy. The therapy involved two operations for par-tial removal, interrupted radiotherapy up to 12Gy, 2 cy-cles of salvage chemotherapy, followed by an operation for total removal, and then a further 3 cycles of che-motherapy. CT scan after the preoperative salvage che-motherapy showed remarkable shrinkage of the re-sidual tumor, which made operative procedure easier.The patient was disease-free for ten months after the last operation.

Patients with combined preoperative chemotherapy and total resection seem to enjoy prolonged progres-sion-free survival, so preoperative chemotherapy for choroid plexus carcinoma seems to be of benefit, des-pite the fact that its optimal recipe has not yet been established.