血管周上皮样细胞肿瘤的临床病理特征及疗效分析

Clinicopathological features and efficacy of perivascular epithelioid cell tumor

目的分析血管周上皮样细胞肿瘤(PEComa)的临床病理特征,以及mTOR抑制剂治疗晚期PEComa的疗效。方法回顾性收集2007—2020年间复旦大学附属肿瘤医院诊断并治疗的17例PEComa患者信息。实体瘤疗效评价标准1.1版评价疗效,Kaplan-Meier法生存分析。结果PEComa主要发生在中年女性,最常见发病部位包括盆腔、肺及腹腔,根治术后复发高峰期为术后2年内。局限期PEComa主要治疗手段为手术切除,对于复发病灶局限者手术切除仍是主要治疗手段。13例晚期恶性PEComa患者接受mTOR抑制剂治疗的客观缓解率为46%,中位无进展生存期为27.6个月(95%CI为4.7~50.5个月)。2例患者分别因肺炎伴呼吸困难及发热终止mTOR抑制剂治疗。结论mTOR抑制剂是治疗晚期恶性PEComa的有效药物,依维莫司相关肺毒性需要关注。

Objective To analyze the clinicopathological characteristics of perivascular epithelioid cell tumor(PEComa)and the efficacy of mTOR inhibitors in the treatment of advanced PEComa.Methods From 2007 to 2020,medical information of 17 patients diagnosed with PEComa and treated in Fudan University Shanghai Cancer Center were collected.Survival analysis was performed by Kaplan-Meier method.Radiological response was assessed according to RECIST v1.1.Results PEComa mostly occured in middle-aged women.The most common primary sites were pelvis,lung and abdomen.Relapse usually occured within 2 years after radical surgery.Radical surgery was the main treatment for PEComa of limited stage,and remains the main option for those with limited recurrent lesions.Of the 13 patients with advanced malignant PEComa in our cohorts,the objective response rate of mTOR inhibitors in advanced malignant PEComa was 46%,and median progression-free survival was 27.7 months(95%CI:4.7~50.5 months).2 patients discontinued mTOR inhibitor treatment due to pneumonitis combined with dyspnea and fever respectively.Conclusions For advanced malignant PEComa,mTOR inhibitors are effective.Lung toxicity of mTOR inhibitor should be monitored.