肺良性转移性平滑肌瘤病7例并文献复习  

Pulmonary benign metastasizing leiomyoma:a report of 7 cases and literature review

作　　者：吴霖 石晨曦[2] 童星 WU Lin;SHI Chenxi;TONG Xing(Department of Thoracic Surgery,The First Affiliated Hospital of Soochow University,Suzhou 215006,Jiangsu,China;Department of Pathology,The First Affiliated Hospital of Soochow University,Suzhou 215006,Jiangsu,China)

机构地区：[1]苏州大学附属第一医院胸外科,江苏苏州215006 [2]苏州大学附属第一医院病理科,江苏苏州215006

出　　处：《山东大学学报（医学版）》2024年第4期78-84,共7页Journal of Shandong University:Health Sciences

摘　　要：目的探讨肺良性转移性平滑肌瘤(pulmonary benign metastasizing leiomyoma,PBML)的临床病理学特征、免疫表型、发病机制、疾病鉴别及诊疗。方法选取2015年8月至2022年1月苏州大学附属第一医院病理科7例PBML的病例和标本资料,均为女性,35~62岁,中位年龄为53岁,均有子宫肌瘤手术史,采用胸部CT、组织病理学分析、免疫组化等方法确诊,分析其影像学、病理学特征、免疫表型,并检索文献对此疾病的发生机制、诊断、疾病鉴别和治疗进行总结分析。结果影像学资料示肺部单发或多发大小不一的类圆形高密度结节。镜下同子宫平滑肌瘤形态。免疫组化指标Desmin、SMA为弥漫的强阳性,ER均为阳性,4例PR阳性,除1例Ki-67增殖指数10%左右,其他1%~5%。S-100、CD117、CD34、HMB45均为阴性。该疾病确诊需结合病史、影像学、病理学及免疫组化,且需要与平滑肌肉瘤、肺纤维平滑肌瘤性错构瘤、炎性肌纤维母细胞肿瘤、孤立性纤维性肿瘤等鉴别。PBML预后一般良好。结论如果患者胸部CT示多发类圆形结节,且患者有子宫肌瘤病史或手术史,则应考虑PBML可能。治疗首选手术切除。Objective To investigate the clinicopathological features,immunophenotype,pathogenesis,differentiation and management of pulmonary benign metastasizing leiomyoma(PBML).Methods The medical records and specimen data of 7 cases of PBML treated in the Department of Pathology,The First Affiliated Hospital of Soochow University during Aug.2015 and Jan.2022 were collected.All patients were female,aged 35 to 62 years(median 53 years),and all had a history of uterine leiomyoma surgery.The diagnosis was confirmed with chest CT,histopathology and immunohistochemistry.The imaging,pathological features and immunophenotypes were analyzed,and relevant literature was collected.Results CT images showed single or multiple quasi-circular hyperdense lesions of varying sizes.PBML was morphologically analogous to uterine leiomyoma.Immunohistochemically,all 7 cases were diffusely and strongly positive for Desmin and SMA.All 7 cases were ER positive,4 cases were PR positive;1 case had Ki-67 proliferation index of 10%,the other 6 had 1%-5%.All cases were negative for S-100,CD117,CD34 and HMB45.The diagnosis of this disease required a combination of history,imaging,pathology,and immunohistochemistry,and needed to be differentiated from leiomyosarcoma,fibroleiomyomatous hamartoma,inflammatory myofibroblastic tumor,and solitary fibrous tumor.The prognosis of PBML was generally favorable.Conclusion If CT images show single or multiple quasi-circular hyperdense lesions of varying sizes and the patient has a history of uterine fibroids or surgery,the possibility of PBML should be considered.Surgical resection is preferred for treatment.

关 键 词：良性转移性平滑肌瘤 罕见肺部疾病 病理特征 免疫组织化学 楔形肺切除 

分 类 号：R655.3[医药卫生—外科学]