颅内上皮样血管内皮瘤的手术治疗:病例报告

A. Gavrjushin, D. Chelushkin
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引用次数: 0

摘要

上皮样血管内皮瘤(EHE)是一种极为罕见的血管肿瘤,其恶性程度介于良性肿瘤和血管肉瘤之间。目前仅发表了 46 例成人原发性 EHE 颅内定位病例:一名 47 岁的男性患者在 3-4 天内出现明显的感觉性语言障碍和失忆性失语,右侧锥体偏瘫。磁共振成像显示患者左侧脑部有局限性胰岛素瘤,伴有出血迹象和病灶周围小水肿。术中,肿瘤由病理血管聚集而成,大脑中动脉 M2 段末端分支受累,因此无法对肿瘤进行根治性切除。根据肿瘤的组织病理学和免疫组化分析(CD31、CD34阳性表达,增殖活性指数Ki-67为10%),确定了EHE的形态学诊断。术后 3 个月,发现肿瘤残余部分继续生长。替莫唑胺化疗临床无效。9 个月后,患者因出现脱位综合征而死亡。EHE的恶性特征包括侵袭性生长、复发和转移,在颅内定位的肿瘤中更为常见。如果怀疑是 EHE,应寻求根治性手术切除肿瘤。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Surgical treatment of intracranial epithelioid hemangioendothelioma: a case report
Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular neoplasm with an intermediate pattern of malignancy between benign neoplasms and angiosarcomas. Only 46 cases of intracranial localization of primary EHE in adults have been published.Case report: A 47-year-old male patient developed pronounced speech disorder of sensory and amnestic aphasia type, right-sided pyramidal hemiparesis within 3–4 days. Magnetic resonance imaging revealed a delimited left sided brain islet neoplasm with signs of hemorrhage and small perifocal edema. Intraoperatively, the neoplasm was represented by a conglomerate of pathologic vessels, with involvement of the terminal branches of the M2 segment of the middle cerebral artery, which prevented radical resection of the neoplasm. Based on histopathological and immunohistochemistry analysis of the neoplasm (positive expression of CD31, CD34, proliferative activity index Ki-67 10%) the morphologic diagnosis of EHE was established. 3 months after surgery, continued growth of the residual part of the neoplasm was noted. Temozolomide chemotherapy was clinically ineffective. The patient died due to the development of dislocation syndrome after 9 months.Discussion. The malignant characteristics of EHE include invasive growth, recurrence, and metastasis, which is more common in intracranial localization of the neoplasm. If EHE is suspected, radical surgical removal of the neoplasm should be sought.
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