肺良性转移性平滑肌瘤机制起源及诊治探讨  

The Origin,Diagnosis and Treatment of Benign Metastatic Leiomyoma of the Lung

作　　者：段端 胡丽娜[1] 刘帅斌 DUAN Duan;HU Li-na;LIU Shuai-bin(Chongqing Medical University,Second Clinical College,Chongqing,400016 China)

机构地区：[1]重庆医科大学第二临床学院,重庆400016

出　　处：《中外医疗》2020年第11期192-195,共4页China & Foreign Medical Treatment

摘　　要：肺良性转移性平滑肌瘤(pulmonary benign metastasizing leiomyoma,PBML)是临床上十分罕见的疾病,好发于绝经前女性,患者常有子宫肌瘤病史或子宫肌瘤手术史;临床表现症状及体征较轻,病程发展缓慢且预后良好。CT表现常见双肺内多发结节,结节多呈轻度强化或无强化,PET-CT结节通常无明显的高代谢摄取。病理特点似成熟的平滑肌细胞,雌孕激素受体常为阳性。临床上需与肺部原发性平滑肌瘤、肺原发性或转移性恶性肿瘤等相鉴别。PBML发病机制存在多种假说,肺和子宫病变在组织病理学、免疫组化和细胞遗传学方面的重叠提示其相关性。PBML治疗方式多以手术治疗为主,结合内分泌治疗常取得不错的治疗效果。酪氨酸激酶抑制剂、下腔静脉滤网植入预防原发病灶转移至肺部等可能对PBML治疗及预防有效。而基因测序PTEN抑癌基因,可提示内分泌治疗尤其是他莫昔芬的耐药性,使用内分泌治疗联合PI3K通路抑制剂克服这种现象,避免非有效治疗的可靠方法。该文将对PBML的临床特点,诊断及鉴别诊断、疾病起源、发病机制及治疗做一综述,旨在为疾病诊断及治疗提供资料依据。Pulmonary benign metastasizing leiomyoma(PBML)is a very rare clinical disease.It occurs in premenopausal women.Patients often have a history of uterine fibroids or a history of uterine fibroid surgery.Clinical symptoms and signs are mild,the course of the disease is slow and the prognosis is good.CT manifestations are often multiple nodules in the lungs,and the nodules are usually mildly enhanced or unreinforced.PET-CT nodules usually do not have significant high metabolic uptake.The pathological characteristics are similar to mature smooth muscle cells,and estrogen and progesterone receptors are often positive.Clinically,it needs to be distinguished from primary leiomyoma of the lung,primary or metastatic malignant tumor of the lung.Multiple hypotheses exist in the pathogenesis of PBML,and the overlap of lung and uterine lesions in histopathology,immunohistochemistry,and cytogenetics suggests their relevance.The treatment of PBML is mainly surgical,and it often achieves good results in combination with endocrine therapy.Tyrosine kinase inhibitors and inferior vena cava mesh implantation to prevent metastasis to the lung may be effective in the treatment and prevention of PBML.Gene sequencing of PTEN tumor suppressor genes may suggest resistance to endocrine therapy,especially tamoxifen.Endocrine therapy combined with PI3K pathway inhibitors is a reliable method to overcome this phenomenon and avoid ineffective treatment.This article will review the clinical characteristics,diagnosis and differential diagnosis,disease origin,pathogenesis and treatment of PBML,in order to provide data basis for disease diagnosis and treatment.

关 键 词：良性转移性平滑肌瘤 子宫平滑肌瘤 转移性肺肿瘤 

分 类 号：R734[医药卫生—肿瘤]