肺良性转移性平滑肌瘤病七例临床分析

The clinical characteristics of pulmonary benign metastasizing leiomyoma

目的 通过分析总结肺良性转移性平滑肌瘤病（PBML）的临床资料,提高临床医生的认识水平并探讨其最佳治疗方案.方法 回顾性分析北京协和医院明确诊断的7例PBML患者的临床资料.结果 7例均为女性患者,中位年龄44（28 -62）岁.4例无症状;3例有症状,气短2例,胸痛1例,紫绀1例,咳嗽1例,痰血1例.6例有20个月- 14年子宫平滑肌瘤病史,5例曾行妇科手术.胸部CT表现为双肺随机分布的多发圆实性结节、双肺弥漫分布粟粒样结节、肺内单发结节以及肺内空洞或环形结节.左锁骨上淋巴结转移1例,右心受累1例.胸腔镜下肺组织活检4例,右心占位切除1例,支气管镜下肺组织活检1例,CT引导下肺穿刺1例.病理表现为交织成簇的饱满的梭形平滑肌细胞形成边界清楚的实性结节,无核多形性、核分裂或坏死,免疫组化均有雌激素受体和孕激素受体阳性.3例药物治疗,来曲唑和诺雷得治疗各1例,结节无缩小;米非司酮治疗1例,疗效不详.1例肺内单发结节者行结节切除术;2例行双附件切除术,术后肺部结节均略有缩小.1例随诊10年病情无进展;1例粟粒性肺结节患者于半年内死亡.结论 PBML与子宫平滑肌瘤相关,需与恶性转移瘤鉴别,临床进展缓慢且预后良好,手术对可切除疾病疗效肯定,促性腺激素疗效有限.对病情稳定者可采取严密临床观察.

Objective Pulmonary benign metastasizing leiomyoma （PBML） is a rare entity that leiomyoma of uterus metastasized to the lung.The clinical characteristics of this rare disease were analyzed in this article.Methods The detailed clinical records of 7 patients diagnosed as PBML at Peking Union Medical College Hospital between January 2001 and June 2015 were reviewed.Results All patients were women with median age of 44 years （range 28-62）.Symptoms included dyspnea （2/7）,chest pain （1/7）,cyanosis （1/7）,cough （1/7） and bloody sputum （1/7）,while 4/7 cases were asymptomatic.Six patients had the past-history of leiomyoma of uterus 20 months to 14 years ago among whom 5 patients received hysterectomy.Chest CT showed bilateral,random-distributed multiple round solid nodules,or diffuse-distributed miliary nodules,or single solid nodule,even some small cavities.Extra-pulmonary metastasis was found in left superclavicular lymph node （1 case） and right heart （1 case）.Histological tissues were obtained by video-assisted thoracic surgery lung biopsy （4/7）,mass resection on tricuspid valve （1/7）,transbronchil lung biopsy （1/7）,and CT-guided percutaneous lung biopsy （1/7）.Pathology showed an interlacing pattern by spindle cells having elongated nuclei without cellular atypia.Ki-67 index was less than 1%.Molecules such as smooth muscle antibody,estrogen receptor （ER） and progestrone receptor （PR） were positive in immunohistochemistry staining.Neither letrozole nor zoladex was effective.Two patients responded to bilateral adnexectomy,presenting as shrunk nodules.No relapsed disease was seen in one patient with single nodule after resection.There was only one patient with disease-related mortality,whose chest CT showed milliary nodules.Conclusion Although CT findings of PBML are similar to malignancies,the clinical outcome is good.Despite the positive expression of ER and PR,the effectiveness of hormone related treatment is limited.And periodical follow up is suggested even to those uneventful patients.