伴纵隔B3型胸腺瘤的肺上皮样血管内皮瘤临床病理观察

Pulmonary epithelioid hemangioendothelioma associated with type B3 thymoma

目的提高对肺上皮样血管内皮瘤(PEHE)临床病理特征以及遗传学特点的认识。方法应用HE染色、免疫组化方法对伴纵隔B3型胸腺瘤的临床病理特征、免疫表型进行分析,并结合文献讨论。结果 PEHE好发于中年女性,临床无特异性症状,影像学检查表现为肺内多发或孤立性病灶;镜下显示黏液样或玻璃样变的背景中瘤细胞呈短索状或小巢状排列,可见胞质内空泡;肿瘤细胞表达血管内皮标记。结论肺上皮样血管内皮瘤少见,无特异性临床表现,诊断主要依靠组织病理学和免疫组化检查。

Purpose To observe and comprehend the pathological and genetic features of pulmonary epithelioid hemangioendothelioma（PEHE）.Methods By using HE stain,immunohistochemistry,and revieweing the related literatures,the characteristics of the clinicopathology and immunophenotype were analysed in one case of pulmonary epithelioid hemangioendothelioma associated with type B3 thymoma.Results PEHE were predominant in the middle-aged women.They presented nonspecial clincal features.Almost all tumors showed multiple or solitary mass in imaging.Histologically,PEHE was composed of rounded or polygonal epithelial-like cells that often contained vacuoles representing intracellular lumina.The tumor cells arranged in short strands or solid nests embedding in a prominent myxochondroid or hyaline stroma.Immunohistochemically,all tumors were positive for at least one endothelial marker.Conclusion PEHE is very rare low-grade malignant tumor,and it has no specific clinical characteristics.The key to diagnosis is histopathological and immunohistochemical study.