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
吴霖 1石晨曦 2童星2
作者信息
- 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
- 折叠
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.
Abstract
Objective To investigate the clinicopathological features,immunophenotype,pathogenesis,differentiation and management of pulmonary benign metastasizing leiomyoma(PBML).Methods The medical records and speci-men data of 7 cases of PBML treated in the Department of Pathology,The First Affiliated Hospital of Soochow Univer-sity 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 im-munohistochemistry.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 qua-si-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Key words
Benign metastasizing leiomyoma/Rare pulmonary disease/Pathological features/Immunohistochemistry/Wedge resection of lung引用本文复制引用
出版年
2024
NETL
NSTL
万方数据