骨原发性上皮样血管肉瘤3例临床病理分析  

Intraosseous epithelioid angiosarcomas,clinical and pathologic analysis of three cases and review of literature

作　　者：张亚青[1] 杨守京[1] 晏伟[1] 徐玉乔[1] 

机构地区：[1]第四军医大学西京医院病理科,陕西西安710033

出　　处：《现代肿瘤医学》2007年第8期1163-1166,共4页Journal of Modern Oncology

摘　　要：目的:探讨3例骨原发性上皮样血管肉瘤的临床病理特点、诊断和鉴别诊断。方法:对3例发生于骨的上皮样血管肉瘤进行光镜、组织化学和免疫组化观察,并结合文献讨论。结果:3例肿瘤分别发生在颅骨、肋骨和骨盆,镜下肿瘤由具有上皮样特征的瘤细胞构成,细胞体积大,胞质丰富带有细小空泡,细胞核大,呈空泡状或受挤压呈印戒样,有明显核仁,核分裂象多见。瘤细胞在骨小梁间呈弥漫片状、腺管状、乳头状或单排条索状排列,有散在完整的红细胞或红细胞碎片的背景,可见形状不规则、大小不一互相交织的血管腔,其内衬上皮样瘤细胞,有的呈乳头状生长。网织纤维染色可清晰的显示瘤性血管结构,并见网织纤维围绕瘤细胞巢分布。免疫组化染色,肿瘤细胞呈上皮性标记物CKpan、高分子量角蛋白、低分子量角蛋白、EMA、间质标记物vimentin以及血管内皮细胞标记物vWF阳性,CD34、CD31弱阳性(考虑因脱钙导致该抗原严重丢失)。结论:骨原发性血管肉瘤是一种罕见的高度恶性肿瘤,诊断和鉴别诊断主要依赖其病理形态学特点结合免疫表型。Objective：To investigate the clinical and pathologic features of three cases of intraosseous epithelioid angiosarcoma （EA）. Methods： Three cases of EA of bone were studied by light microscopy, histochemistry and immunohistochemistry. The pathologic features were discussed with the review of literature. Results： The three osteolytic lesion were demonstrated radiologically in the skull, rib and pelvis. Microscopically, the resected tumors were composed mostly of proliferating malignant ceils with an epithelioid morphology. They had abundant eosinophilic cytoplasm and large nuclei with open chromatin and prominent eosinophilic nucleoli, arranged in a predominantly sheet - like, and an occasional pseudoglandular patterns, mimicking an adenocarcinoma. The dilated anastomotic vascular spaces lined by epithelioid endothelial cells and the intracytoplasmic lumina that sometimes contained erythrocytes suggested focal endothelial differentiation. By immunohistochemistry, the neoplastic cells showed positivity for endothelial cell markers （CD31, CD34, factor Ⅷ- related antigen）, epithelial markers （cytokeratin and epithelial membrane antigen）, and vimentin. Conclusion： EA of bone is a rare, high - grade sarcoma of vascular origin. The correct diagnosis is based on imaging and pathologic examination as well as immunophenotype labeling.

关 键 词：血管肉瘤 骨肿瘤 诊断 鉴别诊断 

分 类 号：R738.1[医药卫生—肿瘤]