背景与目的:原发于中枢神经系统的上皮样血管内皮瘤(epithelioid hemangioendothelioma,EH)非常罕见,本文报道1例天幕起源的EH,并结合文献分析其临床病理、影像学特征及治疗和预后情况,旨在提高对此病的认识.方法:对1例确诊为颅内EH患者的临床、病理和影像学特征、诊断与鉴别诊断、治疗和预后进行回顾性分析并进行文献复习.结果:颅内EH是罕见病变,属于间叶性肿瘤,其生物学行为属交界性或不确定性肿瘤,具有一定特征性的影像学表现.肿瘤由排列呈巢状、索状的上皮样细胞组成.免疫组织化学检测结果显示CD31、CD34和FⅧ阳性.手术切除是其主要的治疗方法,其对放疗敏感.EH的预后较好.结论:EH少见,需与脑膜瘤鉴别,其生物学行为具有潜在的侵袭性,建议尽可能完全切除以及密切随访,对于术后残留病灶推荐放疗.%Background and purpose: Primary intracranial epithelioid haemangioendothelioma (EH) was rare. This study was to discuss the clinical pathological features, radiographic findings, treatment and prognosis of primary intracranial EH combined with literature review. Methods: We reviewed a case of EH reported from our hospital, and comprehensively analyzed the related literature. Results: Based on our report and review of the literature, EH is rare and with borderline or uncertain behavior. The original tumors demonstrated specific image features. The tumor usually appeared as a small nest or cords composed of eosinophilic epithlioid or spindled cells.Immunohistochemical assay were positive for endothelial markers CD31, CD34 and FⅧ. EH may be treated with complete surgical resection whenever possible and is sensitive to radiation. The EH has a favorable prognosis. Conclusion: EH has variable malignant potential, and should be differentiated from meningioma. Total resection and close follow-up is recommended. Additional radiotherapy is advised for residual tumors.
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