骨上皮样血管内皮细胞瘤的临床病理特征六例报告  

Clinicopathologic features of epithelioid haemangioendothelioma:6 cases report

作　　者：李兰[1] 田萌萌[1] 孙晓淇[1] 丁宜[1] 黄啸原[1] 

机构地区：[1]北京积水潭医院病理科,100035

出　　处：《中国骨与关节杂志》2015年第7期552-556,共5页Chinese Journal of Bone and Joint

摘　　要：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 explore the clinicopathologic,immunohistochemical features and differential diagnosis of epithelioid haemangioendothelioma（ EHE）.Methods Six cases of EHE were investigated by immunohistochemistry,and clinical and pathologic features were analyzed.Results Four of the 6 cases were male and the other 2 were female.Four cases presented as solitary lesions,locating in the right acetabulum,the eighth thoracic vertebra,the distal right femur and the sacrum repectively.Two cases presented as multifocal,one of which affected the left acetabulum and the both proximal femur,the other one affected 3 sites of the left proximal femur.The size of the tumor was 1.5-6.0 cm.One case who had a solitary lesion in the right acetabulum recurred 24 months after local excision.Three cases were alive without recurrence after 1,30 and 31 months＇ follow-up.Follow-up was lost in the other two cases.The tumors were composed of epithelioid endothelial cells in a hyalinized,chondroid,or myxoid stroma.There were intracytoplastic lumens（ vacuoles）,but no well-formed vessels.The tumor cells expressed positive at least two of endothelial markers（ include CD31,CD34,FLI-1,and Factor VIII）（ 6 / 6）.Local expressed CK（ 5 / 6）,expression of SMA showed dense minute blood formation.The Ki-67 index was 3%-25%.Conclusions EHE of bone is a rare type of low-to intermediate-grade malignant angiomatous tumors with distinct clinicopathologic features.Typical manifestation：epithelioid endothelial cells were arranged in cords or small nests distributing in hyaline or myxoid stroma.Immunohistochemistry of endothelial markers may be useful for the diagnosis and differential diagnosis.

关 键 词：肿瘤 血管组织 骨肿瘤 病理学 临床 诊断 鉴别 

分 类 号：R738[医药卫生—肿瘤]