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目的探讨骨上皮样血管内皮细胞瘤(epithelioid haemangioendothelioma,EHE)的临床病理特征,诊断及鉴别诊断。方法分析6例骨EHE患者的临床和病理特征,并对其组织标本进行免疫组化标记。结果 6例EHE中男4例,女2例,年龄16~72岁;4例为单发病灶,分别位于右髋臼、第8胸椎、右股骨远端和骶骨;2例为多发病灶。2例多发者中1例3处病灶分布于左髋臼及双股骨上端。另1例3处病灶均位于左股骨近端,病变最大径1.5~6.0 cm。4例行病灶切除,其中1例病变位于右髋臼的患者随访24个月复发,另3例分别随访1个月、30个月和31个月均无复发。2例穿刺活检明确诊断后未行治疗,失访。EHE肿瘤细胞上皮样或梭形,排列成条索状、小巢状,分布于玻璃样变或黏液样变类似软骨基质的间质中,分化良好的血管腔结构少见。免疫组化染色结果显示肿瘤细胞至少有两种血管内皮标记物(包括CD31、CD34、Fli-1和Factor VIII)为阳性(6/6),局灶表达CK(5/6),SMA染色显示在肿瘤内有较多的密集小血管形成,Ki-67抗原标记指数为3%~25%。结论骨原发EHE是较少见的血管源性低-中度恶性肿瘤,典型者表现为上皮样的血管内皮细胞排列成条索状或小巢状分布于玻璃样变或黏液样变的间质中,血管内皮细胞标记物有助于诊断与鉴别诊断。
Objective To investigate the clinicopathological, diagnostic and differential diagnosis of epithelioid haemangioendothelioma (EHE). Methods The clinical and pathological features of 6 patients with bone EHE were analyzed, and the histological specimens were immunohistochemically labeled. Results There were 4 males and 2 females in 6 cases of EHE, ranging in age from 16 to 72 years old. Four of them were single lesion, located in the right acetabulum, the eighth thoracic vertebra, the distal femur and the sacrum respectively. Two cases were multiple lesions. One case of 2 cases with multiple lesions were located in the left acetabulum and the upper femur. Another case of 3 lesions are located in the proximal femur, the largest diameter of 1.5 ~ 6.0 cm. 4 cases were resected, of which 1 case was located in the right acetabulum. The patients were followed up for 24 months and the other 3 cases were followed up for 1 month. There was no recurrence in 30 months and 31 months. 2 cases of biopsy did not confirm the diagnosis after treatment, lost. EHE tumor cells epithelial or spindle-shaped, arranged in a cord-like, small nest, distributed in the glassy or mucoid like cartilage stroma interstitial, well-differentiated vascular cavity structure rare. Immunohistochemical staining showed that at least two vascular endothelial markers (including CD31, CD34, Fli-1 and Factor VIII) in tumor cells were positive (6/6), CK (5/6) In the tumor more dense small blood vessels, Ki-67 antigen labeling index of 3% to 25%. Conclusions The bone primary EHE is a rare type of angiogenic low-moderate malignant tumor. The typical cases are epithelial-like vascular endothelial cells arranged in the shape of cords or small nests distributed in the glassy or mucinous Quality, vascular endothelial markers contribute to the diagnosis and differential diagnosis.