Von Recklinghausen病に合併した多発性脳内悪性神経鞘腫の1例

Multiple Malignant Intracerebral Schwannomas in von Recklinghausen's Disease:Report of a case

I．はじめに

　脳内に発生する神経鞘腫は極めて稀で，Gibsonら^7）（1966）の報告が最初であり，現在までに9例^{6,7,11,12,14，15,16,19）}が報告されているにすぎない．また，本邦での報告は見られない．これら9例の報告はすべて単発性で他の合併症は認めていない．

　数多くのvon Recklinghausen病の報告のなかにも，脳内に発生した神経鞘腫を認めたものはない．神経鞘腫の悪性転化はよく知られているが，頻度はあまり多くない^1）．またmalignant schwannomaの脳転移の報告も見られない．

Intracerebral schwannoma is very rare, and only ninecases of solitary intracerebral schwannoma have been report-ed since the first description by Gibson et al, in 1966. Thisreport is the first case of multiple intracerebral schwannomasin Japan. Intracranial extracerebral schwannomas in vonRecklinghausen's disease are sometimes encountered, butintracerebral ones have not been discovered.

A case of multiple intracerebral schwannomas associatedwith von Recklinghausen's disease is reported in a 23-year-old male. He had been healthy until January 1979 whenhe was pointed out an abnormal shadow on chest roentgeno-gram.