原发性肺动脉内膜肉瘤一例  

A case of primary pulmonary artery intimal sarcoma

作　　者：方珍 乔树叶 以敏 FANG Zhen;QIAO Shu-ye;YI Min(Department of Pathology,Liuzou Workers Hospital,Liuzhou 545007,Guangxi,CHINA)

机构地区：[1]柳州市工人医院病理科,广西柳州545007

出　　处：《海南医学》2024年第19期2853-2856,共4页Hainan Medical Journal

基　　金：广东省柳州市科技计划项目(编号:2021CBB0109);广西壮族自治区卫生健康委员会自筹课题(编号:Z20210819);广西壮族自治区卫生健康委员会自筹课题(编号:Z-B20231374)。

摘　　要：动脉内膜肉瘤是一种罕见恶性间叶肿瘤,其缺乏特征性的临床表现及影像学特点,极易误诊。该肿瘤目前以手术治疗为主,生存期短,预后差。本病例发生于肺动脉,术前超声提示肺动脉栓塞,CT提示肺动脉及其分支充盈缺损或闭塞;术后病理显示肿瘤与动脉内壁关系密切,细胞呈束状或不规则排列,细胞梭形或上皮样,异型性明显,可见瘤巨细胞、坏死及病理性核分裂象,免疫组化显示肿瘤细胞主要表达Vimentin、SMA,荧光原位杂交(FISH)检测显示MDM2基因扩增,病理诊断为肺动脉内膜肉瘤。Artery intimal sarcoma is a rare malignant mesenchymal tumor that lacks characteristic clinical manifestations and imaging features and is easily misdiagnosed.The tumor is currently treated mainly with surgery,with a short survival period and poor prognosis.This case occurred in the pulmonary artery.Preoperative ultrasound showed pulmonary embolism,and CT showed filling defects or occlusion of the pulmonary artery and its branches.Postoperative pathology showed that the tumor was closely related to the inner wall of the artery.The cells were arranged in bundles or irregularly.The cells were spindle-shaped or epithelial-like with obvious atypia.Giant tumor cells,necrosis,and pathological nuclear division were observed.Immunohistochemistry showed that the tumor cells mainly expressed Vimentin and SMA.Fluorescence in situ hybridization(FISH)showed MDM2 gene amplification.The pathological diagnosis was pulmonary artery intimal sarcoma.

关 键 词：肺动脉内膜肉瘤 病理学特征 免疫组化 MDM2基因 

分 类 号：R734.2[医药卫生—肿瘤]