Lhermitte-Duclos diseaseとの併発が疑われたatypical meningiomaの1例

A Case of Atypical Meningioma with Lhermitte-Duclos Disease

Ⅰ．はじめに

　Lhermitte-Duclos diseaseは，小脳半球に限局した腫瘤を形成し，緩徐な発育を示す稀な疾患として知られている⁵⁾．MRIでは肥厚したfoliaが平行に，線状に描出されるparallel linear striationといった特徴的な所見を有し，術前診断も容易になってきている^{3,8,12,15,19,21)}．近年，種々の身体奇形を合併し，常染色体遺伝疾患であるCowden syndrome（multiple hamartoma syndrome）⁷⁾の一部としても位置づけられており，皮膚と粘膜の過誤腫，内臓臓器の過誤腫，顔面頭蓋骨異常，悪性腫瘍の合併が特徴されている^{1,11,14,20,21)}．今回， Lhermitte-Duclos diseaseとの併発が疑われたatypical meningiomaの1例を経験したので，若干の文献的考察を加えて報告する．

Lhermitte-Duclos disease is known as an uncommon disease that characterized by a slowly progressive tumor of the cerebellar hemisphere. We present a case of atypical meningioma with Lhermitte-Duclos disease. A 57-year-old female was admitted to our hospital after presenting with general convulsion. CT scan on arrival showed a large mass of the left frontal region and slightly high-density linear lesion in the right cerebellar hemisphere. MRI also revealed well-enhanced mass in the left frontal region and parallel linear striation in the right cerebellar hemisphere. But,the patient had neither cerebellar dysfunction nor signs of increased intracranial pressure. The patient had angioma of the left breast and bilateral benign struma,no typical manifestation of Cowden syndrome. Removal of the frontal tumor caused the convulsion was subsequently performed. The pathological examination was atypical meningioma. After the operation,radiation therapy was not done because of the total removal of tumor and intension on patient side. Fortunately,the patient had no further adverse neurological events postoperatively. However,we should continuously take account to not only the recurrence of meningioma but also the enlargement of the cerebellar lesion and the complication of malignant tumors in whole body.