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目的探讨颅内多发的脑膜上皮样血管内皮瘤(MEHE)的临床特点、诊断和鉴别诊断。方法回顾性分析1例中枢神经系统原发性脑膜上皮样血管内皮瘤的临床表现、影像学特点及病理组织学形态,通过免疫组化染色进一步确认肿瘤的血管内皮起源。结果患者临床主要表现为发作性头部胀痛,持续数小时可自行缓解,偶有恶心、呕吐,病程长达2年。头部CT及MRI显示枕骨、双侧顶骨及右乳突多发占位,肿瘤推挤压迫枕叶和小脑。组织病理学示在纤维、脂肪组织中见巢状、上皮样分布的肿瘤组织,局部见坏死,部分区域富于血管;肿瘤细胞为卵圆形、短梭形及梭形,胞质丰富、红染,部分胞质空亮,泡状细胞核,核分裂偶见(1个/10 HPF);病理形态非常类似转移性腺癌。免疫组化示肿瘤细胞AE1/AE3、EMA、GFAP、S-100、ER、PR均(-),CK7局灶(+),vimentin、CD31、CD34、FⅧ均(+),Ki-67增殖指数10%。患者术后未做进一步治疗,随访28个月生活能自理,偶有头痛、头晕等不适。结论 MEHE是一种罕见的颅内肿瘤,属于低度恶性,病理极易误诊为转移癌;完整手术切除是治疗首选,放化疗效果有待探讨。
Objective To investigate the clinical features, diagnosis and differential diagnosis of intracranial meningeal epithelioid hemangioendothelioma (MEHE). Methods The clinical manifestations, imaging features and histopathology of primary meningeal endothelium-like hemangioendothelioma in the central nervous system were retrospectively analyzed. The origin of the vascular endothelial cells was further confirmed by immunohistochemical staining. Results The main clinical manifestations of patients with paroxysmal head pain, sustained several hours to ease itself, occasionally nausea, vomiting, duration of up to 2 years. Head CT and MRI showed the occipital bone, bilateral parietal and right mastoid multiple occupy, tumors pushed oppression occipital and cerebellum. Histopathology showed that nests and epithelial-like tumor tissues were found in the fibrous and adipose tissue. The tumors were partly necrotic in some areas and vascular in some areas. The tumor cells were oval, short fusiform and spindle with rich cytoplasm, red Dye, some empty cytoplasm, bubble nuclei, mitotic occasionally (1/10 HPF); pathological morphology is very similar to metastatic adenocarcinoma. Immunohistochemistry showed that the expression of AE1 / AE3, EMA, GFAP, S-100, ER, PR, CK7, vimentin, CD31, CD34, 10%. Patients without further treatment after surgery, 28 months follow-up life can take care of themselves, occasionally headache, dizziness and other discomfort. Conclusions MEHE is a rare intracranial tumor, which belongs to low grade malignancy. The pathology is easily misdiagnosed as metastatic cancer. Complete surgical resection is the first choice of treatment and the effect of radiotherapy and chemotherapy remains to be explored.