肺原发性黏液样肉瘤的临床病理分析及文献复习  

Primary pulmonary myxoid sarcoma:a clinicopathologic analysis and review of literature

作　　者：李杨 郭昆亮[2] 喻朝霞[3] 黄献 胡泉泉 宣兰兰[1] LI Yang;GUO Kun-liang;YU Zhao-xia;HUANG Xian;HU Quan-quan;XUAN Lan-lan(Department of Pathology,Anqing Municipal Hospital,Anqing 246000,China;Department of Cardiothoracic Surgery,Anqing Municipal Hospital,Anqing 246000,China;Department of Pathology,Anqing First People s Hospital,Anqing 246000,China)

机构地区：[1]安庆市立医院病理科,安徽安庆246000 [2]安庆市立医院心胸外科,安徽安庆246000 [3]安庆市第一人民医院病理科,安徽安庆246000

出　　处：《诊断病理学杂志》2025年第2期157-161,共5页Chinese Journal of Diagnostic Pathology

基　　金：安庆市2023年度医疗卫生类科技计划项目(编号:2023Z1002)。

摘　　要：目的探讨肺原发性黏液样肉瘤(PPMS)临床病理学特征。方法收集2015年3月至2024年3月安庆市立医院2例、安庆市第一人民医院1例,共计3例肺原发性黏液样肉瘤,分析临床病理学特征、免疫表型、分子遗传学特征,随访治疗及预后,并系统性复习文献。结果患者男性1例,女性2例,年龄24~72岁。2例无临床症状,体检发现肺肿物。1例无明显诱因出现咳嗽、痰中带血。2例肿瘤位于右肺上叶,1例位于右肺下叶。2例肿瘤与支气管关系密切,1例与支气管不相关,紧贴胸膜。镜检见肿瘤呈多结节状,多角形或短梭形瘤细胞排列成网状、条索状,间质富于黏液样基质。免疫表型:免疫组织化学表达波形蛋白(3/3),上皮膜抗原(EMA)(3/3)、S-100(1/3),Ki-67阳性指数为3%~15%。2例FISH检测存在EWSR 1基因易位,1例NGS检测存在EWSR1-CREB1基因融合。1例行右肺下叶切除术,2例行右肺楔形切除,随访时间5~112个月,3例均无瘤生存。结论PPMS是一种罕见的肺软组织肿瘤,诊断主要依靠发生部位和组织病理学特征,尤文肉瘤断裂区域1基因相关易位检测可有助于诊断。Objective To investigate the clinicopathological features of primary pulmonary myxoid sarcoma(PPMS).Methods Three patients with primary pulmonary myxoid sarcoma(2 cases in Anqing Municipal Hospital and 1 case in Anqing First People s Hospital)were collected from March 2015 to March 2024.The clinicopathological features,immunophenotype,molecular genetic characteristics,follow-up treatment and prognosis were analyzed,and the literature was systematically reviewed.Results Three patients were enrolled in this study,including 1 male patient and 2 female patients,with an age range of 24 to 72 years.Two cases showed no clinical symptoms,but lung masses were found during physical examination.One case presented with cough and blood in sputum without obvious cause.Two cases of tumors were located in the upper lobe of the right lung,and one case was located in the lower lobe of the right lung.Two cases of tumors were closely related to the bronchus,while one case was unrelated to the bronchus and closely attached to the pleura.Microscopically,the tumor was multinodular.Polygonal or short spindle cells arranged in a network or cord like pattern within myxoid stroma.Tumors were immunoreactive for Vimentin(3/3),EMA(2/3),S-100(2/3),SMA(1/3).Ki-67 positive index was 3%-15%.Two cases were shown to harbor EWSR 1 gene rearrangement by FISH.One case had EWSR1-CREB1 gene fusion by NGS.One patient underwent right low lobectomy,and 2 patients underwent right wedge resection.All patients were disease-free after 5-112 months.Conclusion PPMS is a rare sarcoma with low-grade malignant potential.The diagnosis of PPMS depends mainly on the location and histopathological features of PPMS.EWSR1 gene rearrangement is important for the diagnosis of PPMS.

关 键 词：肺肿瘤 肺原发性黏液样肉瘤 EWSR 1 病理诊断 鉴别诊断 

分 类 号：R734.2[医药卫生—肿瘤]