肺动脉内膜肉瘤临床病理学特征病例报告及文献综述.docVIP

肺动脉内膜肉瘤临床病理学特征病例报告及文献综述 　　[摘要] 目的 探讨肺动脉内膜肉瘤的临床病理学特征。 方法 对1例肺动脉内膜肉瘤患者的临床表现、影像学、组织病理学和免疫组织化学特征进行回顾性分析，并且复习相关文献。 结果 患者男性，37岁。临床表现为反复晕厥，偶有头晕。心脏彩超示：右心增大，右室压增高，肺动脉异常回声伴轻度梗阻。肺部CT增强+三维成像示：肺动脉栓塞。行肺动脉瓣取栓术。显微镜下病变组织呈现三个区域：坏死区、稀疏区和密集区。肿瘤细胞主要呈实性生长，浸润血管内膜达血管外膜；肿瘤细胞大部分为梭形细胞，少数为上皮样细胞，异形性明显；间质以胶原为主，可见骨样基质成分；核分裂多见，坏死明显。免疫组化染色肿瘤细胞Vimentin, CD68阳性；myoD1部分阳性；SMA少量细胞阳性；血管标记物CD31部分区域阳性；CD34和因子 Ⅷ血管内皮细胞阳性；ALK和CK阴性。最终病理诊断为肺动脉内膜肉瘤。 结论 肺动脉内膜肉瘤罕见，预后差。临床上难与慢性血管源性疾病（慢性肺栓塞）鉴别，CT及MRI等影像学检查可辅助诊断。 　　[关键词] 肺动脉；内膜肉瘤；病理学；免疫组织化学 　　[中图分类号] R734.2 [文献标识码] B [文章编号] 1673-9701（2012）02-0123-05 　　 　　Clinicopathological features of primary pulmonary artery intimal sarcoma: a case report and review of literatures 　　WANG Mi GAO Feng CHEN Yupeng 　　Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China 　　[Abstract] Objective To explore the clinicopathological features of pulmonary artery intimal sarcoma. Methods The clinical manifestations, imaging, histopathological and immunohistochemical features were analysed in one case of pulmonary artery intimal sarcoma. Related literatures were reviewed. Results A 37-year-old man presented with recurrent syncope and occasional dizziness. Cardiac ultrasound showed: right heart increases, right ventricular pressure heighten, pulmonary abnormal echo with mild obstruction. CT scan with 3D reconstruction: pulmonary embolism. The patient subsequently underwent a pulmonary valve embolectomy. Microscopically, the tumor can be divided into three areas: area of necrosis, sparse and dense areas, mainly in solid tumor cell growth; invasion reach adventitia, and infiltrated pulmonary artery smooth muscle layer; the tumor was predominantly composed of abundant spindle cells with obvious dysplasia, and the small number of epithelial-like cells; interstitial collagen mainly to be seen with suspected osteoid matrix components; mitotic more common, obvious necrosis. On Immunohistochemical examination, the neoplasm were reactive for Vi