摘要：

研究背景 颅内上皮样血管内皮瘤是一种临床罕见的低度恶性肿瘤。本文回顾分析2 例颅内上皮样血管内皮瘤患者临床资料，复习相关文献，对其临床病理学特征、诊断与鉴别诊断、治疗及预后进行总结。方法与结果 2 例患者分别以脑出血和脑膜瘤术后复发就诊，影像学分别提示左侧额叶血肿和脑膜瘤复发，遂行颅内血肿清除术 + 去骨瓣减压术 + 肿瘤全切除术以及肿瘤次全切除术。术后病理证实为上皮样血管内皮瘤。免疫组织化学染色，肿瘤细胞表达内皮细胞标志物（CD34、凝血因子Ⅷ相关抗原，以及CD31、波形蛋白、Fli-1），不表达细胞角蛋白和上皮膜抗原。1 例术后1 年随访肿瘤无复发；1 例术后辅助放射治疗，3 个月后肿瘤转移。结论 颅内上皮样血管内皮瘤是一种临床罕见的生物学行为介于良恶性之间的血管性肿瘤，症状与体征多与占位效应相关，影像学表现无明显特征性，术前明确诊断困难，难以与其他颅内肿瘤相鉴别，明确诊断依靠组织病理学特征。手术全切除是首选治疗方法，若肿瘤全切除，可定期随访观察；若肿瘤未全切除，术后应辅助放射治疗或药物化疗。

关键词: 血管内皮瘤, 上皮样, 脑肿瘤, 神经外科手术

Abstract:

Background  Epithelioid hemangioendothelioma is an uncommon low-grade malignant tumor with various biological behaviors. This paper retrospectively analyzed the clinical data of 2 cases with epithelioid hemangioendothelioma which were confirmed by histopathological features, and reviewed relevant literatures, so as to summarize clinical and radiological features, diagnosis and differential diagnosis, treatment and prognosis of this disease.  Methods and Results  The tumor in Case 1 was cystic accompanied with hemorrhage. Hematoma clearance, decompressive craniectomy and total removal of the tumor were performed. The tumor in Case 2 was misdiagosed as meningioma firstly, and craniotomy was implemented to remove the tumor. But 8 months later, the tumor relapsed. The tumor was vascularized in operation and subtotally removed with moderate blood loss. Postoperative pathological diagnosis revealed epithelioid hemangioendothelioma in both cases. Immunohistochemical staining showed the tumor cells were positive for CD34, FⅧ RAg in Case 1, while CD31, vimentin (Vim) and Fli-1 in Case 2, and both negative for cytokeratin (CK) and epithelial membrane antigen (EMA). Case 1 was followed up for one year, and no recurrence was found. Case 2 received postoperative auxiliary radiotherapy, but tumor metastasis was found 3 months later. Conclusions  Epithelioid hemangioendothelioma is a unique vascular tumor characterized by proliferation of epithelioid or histiocytoid endothelial cells. The histopathological features and biological behaviors are intermediate between hemangioma and angioscarcoma, and its intracranial occurrence is extremely rare. A preoperative diagnosis is usually difficult because of lacking characteristic clinical and radiological features. Diagnosis mainly depends on histopathology and immunohistochemistry. Gross resection is currently the firstline treatment for these tumors. Otherwise, adjuvant therapies are required. The prognosis of this disease has not yet been well defined.

Key words: Hemangioendothelioma, epithelioid, Brain neoplasms, Neurosurgical procedures