纵隔上皮样血管内皮瘤-附3例报告并文献复习

Mediastinal Epithelioid Hemangioendothelioma:A Clinical Study on 3 Cases and Review of Literature

目的:探讨纵隔上皮样血管内皮瘤的病理特征及诊治。方法:通过对3例纵隔上皮样血管内皮瘤的诊治,复习相关文献,就本病临床表现、病理特征、诊断、治疗及预后进行分析讨论。结果:纵隔上皮样血管内皮瘤临床主要表现为瘤体对邻近脏器的局部压迫及刺激症状。病理学提示瘤细胞呈上皮样或组织细胞样,内含血管腔,核分裂象少见。免疫组化显示CD31、CD34、FⅧRAg呈阳性。结论:纵隔上皮样血管内皮瘤是一类罕见的血管源性肿瘤。临床症状及体征无特异性,诊断依赖于组织病理学,尤其是免疫组化。治疗首选手术。因其具有潜在恶性,术后宜行瘤床区局部放疗。

Objective： To study the clinical and pathological features and treatment of mediastinal epithelioid hemangioendo-thelioma. Methods： By case-study method and literature review, clinical and pathological features, methods of diagnosis, treatment and prognosis of mediastinal epithelioid hemangioendothelioma were discussed in the study. Results ： The clinical manifestations of me- diastinal epithelioid hemangioendothelioma mainly came from the stimulation and compression on the adjacent organs. Histologically, the tumor cell characterized with ＂epithelioid＂ or ＂histiocytoid＂, containing lumen and lack of pleomorphism. Immunohistochemical stain showed that the turnout cells were positive for CD34, CD31, FVIHRAg. Conclusions： Mcdiastinal epithelioid hemangioendothelioma is a rare vascular tumor. Because its lacking of specific clinical features, it is difficult to diagnose which generally needs to rely on pathology, and in particular, immunohistochemistry. Surgery is the first choice therapy for mediastinal epithelioid hemangioendothelioma. Because of its potentially malignant, postoperative radiotherapy should be used for the tumor bed area.