肺良性转移性平滑肌瘤六例分析

Pulmonary benign metastasizing leiomyoma： a study of six cases

目的探讨肺良性转移性平滑肌瘤的临床、影像、病理特征及预后。方法回顾性分析2012年10月至2016年12月收治的6例经病理证实的肺良性转移性平滑肌瘤患者的临床及影像学资料，并结合国内外文献报道进行分析总结。结果6例患者均为女性，年龄32-55（43.80 ± 7.17）岁，2例体检发现，2例胸闷气喘，1例胸痛，1例咯血。肺内转移病灶发生在子宫平滑肌瘤术后1个月至15年。胸部CT表现特征为肺内多发病灶5例，其中3例为随机分布多发肿块及结节，2例为弥漫性粟粒结节，1例单发肿块伴发肺原发性腺癌。1例行PET-CT检查示两肺结节均未见放射性摄取明显增高。2例行CT引导下肺穿刺活检，2例经胸腔镜肺活检术，2例行胸腔镜下肺叶楔形切除术。病理显示肿瘤细胞具有平滑肌细胞的分化特征，免疫组化提示结蛋白、平滑肌肌动蛋白、雌激素受体、孕激素受体表达强阳性。3例给予口服三苯氧胺抗雌激素治疗，随访时间为5个月至5年，4例预后良好，2例不详。结论肺良性转移性平滑肌瘤是一种易发生在育龄期女性的罕见疾病，临床症状不典型，需结合影像学检查，及时取得病理结果确诊，手术联合内分泌治疗有效。

ObjectiveTo investigate the clinical, radiological and pathological characteristics, treatment and prognosis of pulmonary benign metastasizing leiomyoma （PBML）. MethodsThe clinical and image data of 6 patients with PBML confirmed by pathology from October 2012 to December 2016 were retrospectively analyzed, and the related literature was reviewed. ResultsSix cases were female, age was from 32 to 55 （43.80 ± 7.17） years. Two cases were found by physical examination, 2 cases had chest distress and asthma, 1 case had chest pain, and 1 case had hemoptysis. Pulmonary abnormalities were detected between 1 month and 15 years after uterine myomectomy. Chest CT showed that multiple lung nodules or masses were observed in 5 patients, among which random distribution was in 3 cases, diffuse military nodule pattern was in 2 cases, and single mass combined with primary lung adenocarcinoma was in 1 case. Fluorine-18-fluorodeoxyglucose positron emission tomography/CT （18F-FDG PET/CT） of one patient showed no obvious uptake of the maximum standardized uptake value. All patients were confirmed histologically with CT guided lung biopsy （2 cases）, thoracoscopic lung biopsy （2 cases）, and thoracoscopic lobectomy with wedge resection （2 cases）. Tumor cells revealed the characteristics of smooth muscle cell differentiation. Immunohistochemistry showed strong positive express of Desmin, SMA smooth muscle specific markers, estrogen receptor and progesterone receptor. Three patients were treated with oral tamoxifen anti estrogen therapy with follow-up from 5 months to 5 years. Four cases had a good prognosis, and 2 cases were lost in follow-up. ConclusionsPBML is a rare disease that is prone to occur in women of childbearing age. The clinical symptoms are atypical. Imaging examination and pathology are necessary for diagnosis. Surgery combined with endocrine therapy is effective.