肺良性转移性平滑肌瘤9例临床病理分析并文献复习

Pulmonary benign metastasizing leiomyoma: a clinicopathological study of 9 cases with review of the literature

目的总结并探讨肺良性转移性平滑肌瘤（pulmonary benign metastasizing leiomyoma,PBML）的临床、影像、病理及预后特点。方法回顾性分析了本院胸外科收治的9例PBML患者临床和病理资料。结果 9例患者均为女性,发病年龄42~53岁。7例有子宫肌瘤病史,其中6例接受过子宫肌瘤手术;2例无子宫肌瘤病史。1例同时伴有腰椎转移。9例均接受肺楔形切除活检手术,经病理和免疫组化检查诊断为PBML。9例患者均长期生存,术后随访时间42~144个月,平均随访时间80个月,术后患者均未接受进一步治疗。2例患者残余病灶缩小或消失,其余患者无进展。结论 PBML是临床罕见疾病。有子宫肌瘤史的女性肺部结节患者需警惕PBML。手术活检是确诊本病的惟一方法。PBML预后良好,部分患者病变可自行缓解。

Objective To investigate the clinicopathological characteristics, diagnosis, treatment and prognosis of pul-monary benign metastasizing leiomyoma（ PBML） . Method The retrospective analysis were performed on the data of 9 cases with PBML from 1999~2014 in Cancer Hospital of Chinese Academy of Medical Sciences. Result All the 9 pa-tients were female, and the suffering age between 42~53 years. Seven patients had a history of uterine fibroids, 6 from which accepted the uterine fibroid surgery. There was no clear history of uterine fibroids in 2 patients. 1 patient-saccompanied with the lumbar metastasis. The confirmed diagnosis of 9 cases were obtalned by surgical biopsy, the postoperative pathology and immunohistochemistry. With the average follow up time 80 months between 42 ~144 months,all the 9 patients owned a long term survival. The residual nodules in lung were stable and part of which dis-appeared in 2 cases during follow up. Conclusion Pulmonary benign metastasizing leiomyoma is a rare and often misdi-agnosed disease. Doctors should be aware of the possibility of PBML in female patients with pulmonary nodules com-bined by history of uterine fibroids. The only possible method for diagnosis of PBML is by surgical biopsy. The nature of PBML provides a good prognosis, and spontaneous remission may be found in these patients.