重症肌无力伴发胸腺瘤患者胸腺瘤切除术联合放疗、环磷酰胺治疗疗效评价

The clinical outcomes of thymectomy combined with radiotherapy or cyclophosphamide treatment for myasthenia gravis patients with thymoma

目的 评价重症肌无力（MG）合并胸腺瘤患者行胸腺瘤扩大切除术后的疗效.方法 回顾性分析2008年7月-2010年12月解放军第三○九医院收治的178例行胸腺瘤切除术的MG患者资料.患者术后接受单用环磷酰胺免疫治疗或联合放射治疗,通过比较术前和术后1个月、6个月、1年及2年MG严重程度评分,评价患者术后不同阶段的疗效.进一步分析不同胸腺瘤病理分型、手术分期与疗效的相关性.结果 （1）178例伴发胸腺瘤MG患者中,男103例,女75例,年龄（43.7±12.5）岁,术后接受放疗＋免疫治疗58例,单纯免疫治疗112例,5例未接受放疗或免疫治疗,3例未完成整个免疫治疗疗程.（2）接受胸腺瘤切除术的患者术后1个月、6个月、1年及2年的总有效率分别为32.8％（58/177）、59.8％（101/169）、69.7％（115/165）及81.5％（132/162）.且随着时间的延长,MG临床绝对评分逐步下降.（3）胸腺瘤切除术后2年时,按临床疗效分为有效病例及无效病例,有效病例与无效病例的各胸腺瘤病理分型构成情况的差异无统计学意义（P＞0.05）;Masaoka分期Ⅰ期、Ⅱ期患者中有效病例的比例高于Ⅲ期、Ⅳ期（91.4％、89.8％比45.5％、28.6％,P均＜0.001）.结论 伴胸腺瘤的MG患者行胸腺瘤扩大切除术后联合免疫治疗和/或放射治疗,随着时间延长MG症状临床缓解率逐步提高,术后2年有效率可达81.5％.临床疗效与胸腺瘤病理类型无关,但与Masaoka分期相关.

Objective To evaluate the therapeutic effects of thymectomy in myasthenia gravis （MG） patients with thymoma.Methods A total of 178 MG patients with thymectomy between July 2008 and December 2010 were included.All the subjects were received either cyclophosphamide alone or in combination with radiotherapeutic treatment after surgery.The MG absolute and relative clinical scores were used to assess the effectiveness of long-term trcatments.Clinical evaluations were conducted before,and at 1,6,12 and 24 months after operation.A comparative analysis on the inter-relationships among MG clinical presentation,WHO histology aud Masaoka stage was also conducted.Results （1） Of the 178 thymomapatients,103 were male and 75 were female,with a mean age of （43.7 ± 12.5）-years old.One hundred and twelve cases were taken cyclophosphamide,and 58 cases with invasive thymoma （stages Ⅱ,Ⅲ and Ⅳ or WHO type B3） were taken cyclophosphamide in combination with radiotherapy.Five patients refused cyclophosphamide or radiotherapy and 3 did not finish treatment.（2） The muscular strength improved obviously in 32.8% （58/177） of the patients after 1 month after thymectomy,and 59.8% （101/169）,69.7% （115/165） and 81.5% （132/162） after 6 months,1 year and 2 years,respectively with MG score for disease severity decreased significantly with time.（3） No significant differences of the improvement rates were observed in patients within different WHO histology category.However,the rates were much higher in patients with Masaoka stage Ⅰ （91.4%） and stage Ⅱ （89.8%） than those in patients with stage Ⅲ （45.5%） and Ⅳ（28.6%） （all P〈0.001）.Conclusions The remission rate of MG patients with thymomas increase after thymectomy plus cyclophosphamide or in combination with radiotherapy and reached 81.5% after 2 years.The remission rate is associated with Masaoka stagc,but not with WHO histology.