淋巴结原发上皮样血管内皮瘤1例及文献复习

Primary epithelioid hemangioendothelioma of lymph node: one case report and review of the literatures

目的 探讨淋巴结原发上皮样血管内皮瘤的诊断及鉴别诊断。方法 1例淋巴结原发上皮样血管内皮瘤进行HE切片、组织化学、免疫组化等观察 ,并复习文献进行讨论。结果 光镜下肿瘤细胞呈多角形或类圆形上皮样 ,无明显核分裂象 ,组织结构多种 ,呈单个细胞条索、实性巢状及管腔样结构排列 ,瘤细胞形成小的细胞内管腔 ,见胞质内空泡 ,并见红细胞 ,此特征为细胞原始管腔结构。玻璃样间质。网状纤维染色显示血管腔隙结构。肿瘤细胞表达Vim、CD31、FⅧRAg ,少数细胞表达CD34。结论 淋巴结原发上皮样血管内皮瘤是极少见的低度恶性血管性肿瘤 ,可出现多种组织结构 ,有一定的特征性。应与转移性癌、转移性恶性黑色素瘤、上皮样血管肉瘤、上皮样血管瘤。

Purpose To study diagnosis and differential diagnosis of primary epithelioid hemangioendothelioma of lymph node. Methods A case of primary epithelioid hemangioendothelioma of lymph node was investigated by hematoxylin-eosin , histochemical and immunohistochemical staining. Results Microscopically, the neoplastic cells appeared polygonal or oval epithelioid, lacked mitotic activity and arranged in a variety of patterns, including solid nests, single cell cords or luminal patterns. The tumor cells formed small intracellular lumens which were seen as cytoplasmic vacuoles. These miniature lumens occasionally contained erythrocytes. The primitive lumen formation are characteristic appearance. The neoplastic cells were embedded in hyalinized matrix. Vascular channels were highlighted by reticulin staining. The tumor cells expressed vimentin, CD31 and FⅧRAg, a minority of cells expressed CD34. Conclusions Primary epithelioid hemangioendothelioma of lymph node is a rare low-grade angiosarcoma. It is important to recognize its morphological characteristics to avoid confusion with other similar lesions, such as metastatic carcinomas, malignant melanoma, epithelioid angiosarcoma, epithelioid hemangioma, epithelioid sarcoma, and sclerosing epithelioid fibrosarcoma.