肺动脉内膜肉瘤10例临床病理观察  

Pulmonary intimal artery sarcoma: a clinicopathological study of ten cases

作　　者：魏淑飞 邹秋琴 郑涛 张卢舜 侯英勇 WEI Shufei;ZOU Qiuqin;ZHENG Tao;ZHANG Lushun;HOU Yingyong(Department of Pathology,Jiujiang University Affiliated Hospital,Jiangxi Jiujiang 332000,China;Department of Pathology,Zhongshan Hospital,Fudan University,Shanghai 200032,China)

机构地区：[1]九江学院附属医院病理科,江西九江332000 [2]复旦大学附属中山医院病理科,上海200032

出　　处：《临床与实验病理学杂志》2023年第11期1340-1344,共5页Chinese Journal of Clinical and Experimental Pathology

摘　　要：目的 探讨肺动脉内膜肉瘤的临床病理学特征、分子改变、治疗及预后。方法 收集10例肺动脉内膜肉瘤的临床资料,采用HE、免疫组化EnVision法染色并行FISH检测,并复习相关文献。结果 患者男性4例,女性6例,男女比为1∶1.5,年龄33~75岁,平均55.7岁。临床症状以无明显诱因出现胸闷气促(6/10)、胸痛(5/10)、咳嗽(3/10)为主,部分患者出现咯血(2/10)、晕厥(1/10)及心脏杂音(1/10)。3例伴肿瘤病史,1例伴双侧乳腺癌、双侧甲状腺乳头状癌及浸润性肺腺癌,1例双侧乳腺癌,1例肺动脉栓塞及心脏黏液瘤。影像学术前检查示肺动脉栓塞或肺肿瘤。组织学显示肿瘤细胞呈梭形或上皮样,异型性明显,核分裂象易见,2例可见瘤巨细胞;部分向横纹肌肉瘤、血管肉瘤及平滑肌肉瘤分化;缺乏特异性免疫组化标志物,肿瘤细胞可表达vimentin、Fli-1、SMA、MyogD1、Myoglobin、BCL-2、ERG等,Ki-67增殖指数30%~70%。FISH检测MDM2(4/5)、CDK4(1/1)基因扩增。10例均行手术治疗,7例获得随访,随访时间1~17个月,其中4例出现复发或远处转移。结论 肺动脉内膜肉瘤临床罕见,异质性高,免疫组化标志物无特异性且基因改变复杂,无统一治疗方案,患者预后差。Purpose To investigate the clinicopathologic features,molecular changes,treatment and prognosis of pulmonary artery intimal sarcoma.Methods Ten cases of pulmonary artery intimal sarcoma were collected and the clinical features analyzed,by using HE,immunohistochemistry EnVision method,FISH,and review of relevant literature.Results There were 4 males and 6 females,with a male to female ratio of 1∶1.5.The patients were 33-75 years old with an average age of 55.7 years.The main clinical symptoms were chest tightness,shortness of breath(6/10),chest pain(5/10)and cough(3/10),hemoptysis(2/10),syncope(1/10),heart murmur(1/10).1 patient had a history of bilateral breast cancer,bilateral papillary thyroid carcinoma and invasive lung adenocarcinoma,1 patient had bilateral breast cancer and 1 patient had pulmonary embolism and cardiac myxoma.Preoperative imaging showed pulmonary embolism or lung tumor.Histological morphology showed that the tumor cells were fusiform or epithelioid,with obvious atypia.Some tumors differentiated into rhabdomyosarcoma,angiosarcoma and leiomyosarcoma,and giant cells were seen in 2 cases.The tumor lacked specific immune markers,and the tumor cells expressed vimentin,Fli-1,SMA,MyogD1,Myoglobin,BCL-2,ERG,etc.Ki-67 proliferation index was about 30%-70%.Fluorescence in situ hybridization was used to detect MDM2(4/5)and CDK4(1/1).All cases received surgical treatment,7 cases were followed up from 1 month to 17 months,and 4 cases of them had recurrence or distant metastasis.Conclusion Pulmonary artery intimal sarcoma is rare,without specific immune markers and with complicated gene changes.There is no standard treatment,and the prognosis is poor.

关 键 词：肺肿瘤 肉瘤 分子改变 诊断 预后 

分 类 号：R734.2[医药卫生—肿瘤]