結節性硬化症を伴わないsubependymal giant cell astrocytomaの1例

A Case of Subependymal Giant Cell Astrocytoma not Associated with Tuberous Sclerosis

Ⅰ．はじめに

　Subependymal giant cell astrocytoma（SGCA）は，結節性硬化症に随伴し脳室上衣下に特異な病理像を呈する腫瘍である．若年者の側脳室壁に好発し，限局性の脳室内腫瘤として増殖するが，モンロー孔を閉塞し水頭症で発見されることも多い，一方近年，結節性硬化症の徴候を認めないSGCAの報告が散見される^2-9,13-15,17．今回われわれは，結節性硬化症を伴わないと考えられるSGCAの1例を経験したので，若干の文献的考察を加え報告する．

The authors present a case of a subependymal giant cell astrocytoma (SOCA) not associated with tuberous sclero-sis. On admission, a six-year-old boy had obstructive hydrocephalus caused by a huge intraventricular tumor. Preop-erative T1-weighted MR images with gadolinium showed heterogeneous enhancement of the lesion. The tumor was totally removed through a right transcortical approach with frontal craniotomy. Immunohistochemical staining for glial fibrillary acidic protein (GFAP) showed a positive reaction in some of the large tumor cells and the labeling in-dex of MIB-1 was＜1.0%. Histological examinations revealed SGCA.