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目的:探讨肺活体组织检查肺上皮样血管内皮瘤(pulmonary epithelioid haemangioendothelioma,PEH)意义及鉴别诊断。方法:回顾分析4例肺穿刺活体组织检查诊断PEH患者的病理特点、免疫组织化学检查结果、临床及实验室检查结果。结果:4例PEH患者年龄30～61岁,男女比2∶2;影像学检查示肺内结节考虑为转移性癌,实验室检查未见明显异常。光镜下肿瘤细胞有上皮样及空泡样形态,空泡内偶见红细胞,瘤细胞呈条索状、小巢状,腺样结构及不规则状分布于淀粉样或玻璃样变间质中,瘤细胞未见核分裂相,肿瘤边缘区细胞丰富,呈息肉或肾小球样填塞于扩张的肺泡腔。肿瘤细胞表达CD31、CD34、第Ⅷ因子相关抗原(factorⅧrelated antigen,FⅧRAg)等血管内皮细胞标记,同时部分表达波形蛋白(vimentin)和细胞角蛋白(cytokeratin,CK)。结论:肺穿刺活体组织中,如果病变具有典型PEH形态学特征及免疫表型,同时又有临床及影像学支持,即可诊断PEH,应用多组抗体有助于PEH的诊断及鉴别诊断。
Objective: To investigate the significance and differential diagnosis of pulmonary epithelioid haemangioendothelioma (PEH) in lung biopsy. Methods: Retrospective analysis of 4 cases of pulmonary biopsy biopsy diagnosis of pathological features of patients with PEH, immunohistochemistry results, clinical and laboratory test results. RESULTS: Four PEH patients were between 30 and 61 years of age with a male to female ratio of 2:2. Imaging examination showed that nodules in the lung were metastatic cancers. No abnormalities were found in laboratory tests. Under light microscope, tumor cells had epithelioid and vacuolar morphology. Even in vacuoles, red blood cells were seen. The tumor cells showed cords and small nests. The adenoid structure and irregular shape were distributed in the amyloid or hyalinized stroma. There was no mitotic phase in the tumor cells. The cells in the tumor marginal zone were rich in polyps or glomerular filling in the dilated alveolar space. The tumor cells express CD31, CD34, Factor VIII related antigen (FVIIIRAg) and other vascular endothelial cell markers, and partially express vimentin and cytokeratin (CK). Conclusion: In lung biopsy, PEH can be diagnosed if the lesion has typical PEH morphological characteristics and immunophenotype, and there is clinical and imaging support. The application of multiple antibodies can help to diagnose and differentially diagnose PEH.