肺上皮样血管内皮细胞瘤临床病理诊断与鉴别诊断

Clinical Pathological diagnosis of pulmonary epithelioid hemangioendothelioma and differential diagnosis

目的分析肺上皮样血管内皮细胞瘤(PEH)临床病理特点,加强对该疾病的认识,提高诊断水平。方法结合本科诊断的1例PEH患者的临床病理资料和文献复习,分析该病的临床病理特点和鉴别诊断。结果 PEH影像学表现为双肺多发性结节。组织学表现为上皮样细胞构成的结节中心硬化或呈淀粉样变,结节周边富细胞,瘤细胞轻到中度异型,胞质内见原始血管腔。瘤细胞呈巢状伸入周围的肺泡腔生长。免疫组化提示瘤细胞主要表达CD31、CD34、、Ⅷ因子、Vimentin、CK等标记物。鉴别诊断主要包括硬化性血管瘤、上皮样血管瘤、上皮样血管肉瘤、上皮样肉瘤等。结论正确的诊断与鉴别诊断PEH需要结合影像学检查、组织学观察和免疫组织化学分析等方法。

Objective：To describe the clinical pathological charahteristics of pulmonary epithelioid haemangioendothelio-ma（ PEH）and strengthen the understanding of the disease and improve the diagnostic level. Methods：With a case report and review of the related literature,the cliniacal pathologic charahteristics of PEH and differential diagnosis were de-scribed. Results：The imaging characteristic of PEH was muttiple nodules in both sides of the lung. The histological mani-festations of PEH showed a hardening or amyloidosis in the center of the nodule constituted with epithelioid cells,and rich cells aroud the nodule,and mild-moderate atypia of tumor cells,and the original blood vessel lumen in the cytoplasm. The nests of tumor cells grow into the alveolar aroud the nodule. Immunohistochemical analysis showed that tumor cells mainly express CD31,CD34,Ⅷfactor,Vimentin,CK and other markers. Conclusion：Correct diagnosis and differential diagnosis a-bout PEH requires a combination of imaging studies,histological observation and immunohistochemical analysis and other methods.