頭蓋内および脊柱管内に多発性病変を呈したRosai-Dorfman diseaseの1例

Rosai-Dorfman Disease Presenting with Multiple Intracranial and Intraspinal Masses : A Case Report

Ⅰ．はじめに

　Rosai-Dorfman disease（以下RDD）は1969年にRosaiとDorfman^8）によりsinushistiocytosis withmassive lymphadenopathyとして報告された．臨床症状は無痛性両側頸部リンパ節腫脹，発熱，赤沈亢進などを特徴とし，病理所見は成熟した組織球，リンパ球および形質細胞の増殖とリンパ球を貧食した組織球（emperipolesis）を認めるリンパ節の肉芽腫性病変である．節外病変として上気道，副鼻腔，皮膚などにも発生するが，中枢神経系の病変は稀である．今回われわれは，頭蓋内と脊柱管内に多発性の病変を認めたRDDの稀な1例を経験したので文献的考察を加え報告する．

Rosai-Dorfman disease (RDD), a systemic histioproliferative disorder, was first described by Rosai and Dorfman in 1969. However, only 41 cases involving the central nervous system have been reported. The authors present a rare case of RDD with multiple intracranial and spinal lesions mimicking multiple meningioma.

A 59-year-old woman was admitted to our hospital with a history of bilateral visual impairment. MRI demonstrated multiple central nervous system lesions in the suprasellar region, right temporal convexity, left frontal convexity, left cerebello-pontine angle and C5 level of the spinal cord.