骨上皮样血管内皮瘤4例及文献复习  

Epithelioid hemangioendothelioma of bone: a study of 4 cases and review of literature

作　　者：周军[1] 印洪林[1] 张海芳[2] 马捷[1] 余波[1] 金行藻[1] 周晓军[1] 

机构地区：[1]南京大学医学院临床学院/南京军区南京总医院病理科,南京210002 [2]厦门大学附属第一医院病理科,厦门361003

出　　处：《临床与实验病理学杂志》2012年第4期410-414,共5页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的探讨4例骨上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)的临床病理特点、诊断和鉴别诊断。方法对4例原发于骨的EHE临床资料、组织学、免疫组化进行观察分析并随访,结合文献讨论。结果 4例骨EHE中男性和女性各2例,中位年龄24岁,发生部位包括右肩胛骨、腰4～5椎体、胫骨下段和左足。临床症状主要为疼痛,影像学资料提示溶骨性改变;镜下示上皮样的肿瘤细胞呈巢状、索状或小梁状排列,肿瘤细胞呈圆形、多边形间或梭形,常见明显的胞质内空泡并伴有周围间质的玻璃样或黏液样变性。免疫表型:瘤细胞CD31、CD34、α-SMA均阳性(100%),CKpan有1例阳性(25%),EMA均阴性(100%),肿瘤增殖标记Ki-67呈低表达。结论原发于骨的EHE是一种少见的低度恶性的血管源性肿瘤,它的诊断要结合临床和影像学资料,依据其病理学特征及相关的免疫组化特点综合判断,需与骨转移性癌、上皮样血管瘤、上皮样血管肉瘤(epithelioid angiosarcoma,EAS)等鉴别。Purpose To investigate the clinicopathological characteristics,diagnosis and differential diagnosis of epithelioid hemangioendothelioma（EHE） derived from bone.Methods Four cases of EHE in bone were retrospectively reviewed on the clinical data,histological and immunohistochemical features,the follow-up,and the related literature.Results Cases studied consisted of 2 males and 2 females,with median age 24,and the anatomic locations included the right scapular bone,L4 vertebra,left distal tibia,and left foot.The main clinical and radiographic manifestations were ache and lytic lesions,respectively.Histologically,EHE was comprised of the epithelioid cells arranged in nets,cords and strands,usually,with the significant appearance of cytoplasmic vacuoles and the surrounding hyaline and myxoid stroma.Immunohistochemically,tumor cells were positive for CD31,CD34,α-SMA,and partly positive for CKpan（25%） and negative for EMA,in addition to the low expression of Ki-67.Conclusion EHE derived from bone is a rare and low aggressive tumor.A correct diagnosis needs the comprehensive evaluation including the relevant clinical pathology characteristics and immunohistochemistry,and the exclusion of metastatic carcinoma of bone,hemangioma,and epithelioid angiosarcoma.

关 键 词：骨肿瘤 上皮样血管内皮瘤 病理学 诊断 鉴别诊断 

分 类 号：R738.1[医药卫生—肿瘤]