鞍上部肉芽腫に対し開頭摘出術を施行したErdheim-Chester病の1例

A Case Report of Surgical Resection of a Supraseller Granuloma in a Patient with Erdheim-Chester Disease

Ⅰ．はじめに

　Erdheim-Chester（E-C）病は1930年にChester³⁾により初めて報告された稀な疾患で，長管骨に特徴的病的所見を呈する非ランゲルハンス細胞性組織球症の一型である⁴⁾．本症は，非ランゲルハンス細胞系の組織球細胞が異常に増殖し，全身に浸潤し，さまざまな症状を呈する．本症の原因は不明であるが，近年，がん遺伝子であるBRAF遺伝子やNRAS遺伝子などの変異が認められるとの報告がある^6,11,13)．今回われわれは，鞍上部肉芽腫による右視力低下で発症したE-C病例において，肉芽腫の開頭摘出術を行った．E-C病の頭蓋内病変に対し開頭摘出術を行った報告は少なく，文献的考察を含め報告する．

　Erdheim-Chester disease（E-CD）is a rare pathology characterized by systematic granulomatosis that occasionally involves the central nervous system. We report about a 68-year-old woman with E-CD who presented with right-side visual disturbance. Magnetic resonance imaging showed a suprasellar tumor that elevated the right optic nerve and involved the right internal carotid and right anterior choroidal arteries. The tumor was partially resected via a trans-Sylvian approach and was histologically diagnosed as a granuloma. Considering the abnormal findings of postoperative X-ray and 99 mTc bone scintigraphy of the long bones, the pathology was diagnosed as E-CD. After surgery, her right-side visual disturbances disappeared. However, 1 year later, she died of systemic infection and heart failure. Histological autopsy findings indicated numerous yellowish nodules in the heart, lung, and kidney with pericardial and pleural effusions and whole-body granulomatosis, including the brain.

　E-CD is a rare but critical disease. This pathological entity should be considered when encountering cases of intracranial granuloma to ensure its early diagnosis and appropriate treatment. Surgical resection of intracranial granulomas in patients with E-CD may promptly improve neurological dysfunction.