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Neurological Surgery No Shinkei Geka Volume 46, Issue 1 （January 2018）

Neurological Surgery 脳神経外科 46巻1号（2018年1月発行）

Japanese English

症例

頭蓋内に原発したepithelioid hemangioendotheliomaの1例 A Case of Primary Intracranial Epithelioid Hemangioendothelioma 山本福子 ¹ , 山際啓典 ¹ , 岩本文徳 ¹ , 春日井務 ² Fukuko YAMAMOTO ¹ , Hironori YAMAGIWA ¹ , Fuminori IWAMOTO ¹ , Tsutomu KASUGAI ² ¹独立行政法人地域医療機能推進機構大阪病院脳神経外科 ²独立行政法人地域医療機能推進機構大阪病院病理科 ¹Department of Neurosurgery, Japan Community Health care Organization（JCHO）Osaka Hospital ²Department of Pathology, Japan Community Health care Organization（JCHO）Osaka Hospital キーワード： epithelioid hemangioendothelioma , primary brain tumor , convulsion Keyword: epithelioid hemangioendothelioma , primary brain tumor , convulsion pp.35-40

発行日 2018年1月10日 Published Date 2018/1/10

DOI https://doi.org/10.11477/mf.1436203671

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Abstract 文献概要
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参考文献 Reference

Ⅰ．背景

　Epithelioid hemangioendothelioma（EHE）は肺や肝臓，骨，軟部組織などさまざまな臓器に発生する中間悪性の血管内皮性腫瘍であるが，頭蓋内の発生は極めて稀である．今回，われわれは痙攣で発症した頭蓋内原発のEHEを経験したので，文献的考察を加え報告する．

　Epithelioid hemangioendotheliomas（EHEs）most commonly occur in the liver, lung, bone, and soft tissues, but rarely in the brain. Here, we describe a case of primary intracranial epithelioid hemangioendothelioma manifested by an epileptic attack.

　A 53-year-old woman with no previous convulsive history presented at our hospital after experiencing a seizure. Magnetic resonance imaging revealed a mass in her right frontal lobe, with edema, which was well enhanced with gadolinium. Systemic computed tomography, on the other hand, did not show any tumor involvement in other organs. The patient underwent surgery, and a complete resection of the intracranial tumor was successfully performed. The histological diagnosis was an EHE. There was no evidence of tumor recurrence either in the brain or other organs at 3 years and 7 months after surgery.

　Because of its rarity, the exact prognosis of patients with an intracranial EHE has not been clearly established. Moreover, tumor recurrence following complete resection has been reported and thus regular follow-up examinations are considered necessary.