雑誌文献を検索します。書籍を検索する際には「書籍検索」を選択してください。

Neurological Surgery No Shinkei Geka Volume 20, Issue 11 （November 1992）

Neurological Surgery 脳神経外科 20巻11号（1992年11月発行）

Japanese English

症例

Von Recklinghausen病に伴うGliosarcomaの1例 Gliosarcoma Associated with von Recklinghausen's Disease: A case report 合志清隆 ^1,2 , 撫中正博 ¹ , 山田治行 ¹ , 花田貴信 ¹ , 和田伸一 ¹ , 横田晃 ¹ Kiyotaka KOHSHI ^1,2 , Masahiro MUNAKA ¹ , Haruyuki YAMADA ¹ , Takanobu HANADA ¹ , Shin-ichi WADA ¹ , Akira YOKOTA ¹ ¹産業医科大学脳神経外科 ²産業医学大学病院高気圧治療部 ¹Department of Neurosurgery, School of Medicine, University of Occupational and Environmental Health キーワード： Von Recklinghausen's disease , Gliosarcoma Keyword: Von Recklinghausen's disease , Gliosarcoma pp.1195-1198

発行日 1992年11月10日 Published Date 1992/11/10

DOI https://doi.org/10.11477/mf.1436900559

PDF(5450KB)

有料閲覧

Abstract 文献概要
1ページ目 Look Inside

I．はじめに

　Von Recklinghausen病は皮膚を主病変とする遺伝性疾患であるが，種々の脳腫瘍を合併することもよく知られている．合併する脳腫瘍のなかではacoustic neurino—maが最も多く，次いでmeningiomaやgliomaなどが挙げられ，gliomaのなかでは高分化のoptic glimaが多く，malignant gliomaは少ないと言われている^6,14）．

　一方，ghosarcomaはglioblastomaとsarcomaからなり稀なものと考えられていたが，anaplastic astrocyto—maの8％を占めるとするものもあり^13），近年本邦でも10数例報告されている^10）．

A very rare case of gliosarcoma with von Reckling-hausen's disease is presented. A 51-year-old man was admitted to our hospital in March 1990, because of a 2-month history of personality change and left hemi-paresis. Multiple neurofibromata over his whole body with many cafe au lait spots had been present since early childhood. His mother, brothers and children also had cafe au lait spots. Neurological examination on admis-sion revealed memory disturbance, left homonymous hemianopsia and left hemiparesis.

CT scan showed a large lobular lesion in the right term poroparietal region. The medial hypodense part was sharply demarcated by a ring-like enhancement, while the lateral isodense part was homogeneously enhanced. MRI showed a sharply demarcated high intensity lesion with Gd-EDTA enhancement corresponding to the en-hanced area on CT. Faint staining on angiography re-vealed that the blood supply to the tumor was predomi-nantly d ural.

At surgery, the tumor was solid and highly vascular, and adhered tightly to the dura. The superficial part of the tumor was well demarcated from the brain tissue, but the demarcation was obscure in the deeper part.

Histological findings showed two clearly defined neo-plastic components: a gliomatous component that was stained for GFAP, and a sarcomatous component that had spindle-shaped nuclei and eosinophilic fibers. The patient underwent radiotherapy after surgery, but the tumor soon recurred. A second operation was per-formed, but the tumor had infiltrated into the scalp and he died 10 months after the first operation.

This combination is very rare and has not been re ported previously.