結節性硬化症に合併し腫瘍内出血で発症した上衣下巨細胞性星細胞腫の1例

Hemorrhagic Onset of Subependymal Giant Cell Astrocytoma Associated with Tuberous Sclerosis:A Case Report and Review of Literature

Ⅰ．はじめに

　結節性硬化症（tuberous sclerosis：TSC）は遺伝性の神経皮膚疾患で，3.4〜17%に脳腫瘍を合併する^3,23)．その多くは上衣下巨細胞性星細胞腫（subependymal giant cell astrocytoma：SEGA）で，側脳室前角，特にMonro孔近傍に発生する^3,18,19)．今回われわれは腫瘍内出血により急性水頭症を来したSEGAの1例を経験した．本症例では緊急避難的に脳室外ドレナージを行ったが，待機的に開頭腫瘍摘出術を行うまでの間に腫瘍内出血を繰り返した．これまで腫瘍内出血を来したSEGAの報告は極めて少なく，渉猟し得た範囲では12例であった^{1,10,11,13,14,17,19-23)}．過去の報告例と合わせて，腫瘍内出血で発症したSEGAの病態と治療について考察し報告する．

　We report on a case of subependymal giant cell astrocytoma（SEGA）in a patient with tuberous sclerosis（TSC）that presented with intratumoral hemorrhage and acute hydrocephalus. Initial treatment was external ventricular drainage to control the intracranial pressure;however, the tumor increased in size due to recurrent hemorrhage. Subsequently, the tumor was successfully removed via the transcortical-transventricular approach without neurological deterioration. Although intratumoral hemorrhage is extremely rare in patients with SEGA, subsequent acute hydrocephalus resulting from obstruction of the foramen of Monro will be fatal if prompt surgical treatment is not available. Careful and periodical radiographic examination of the central nervous system will be mandatory in patients with TSC, especially in those who have subependymal nodules（SEN）or SEGA around the foramen of Monro. Radical surgical removal should be considered before they become symptomatic.