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目的探讨重症肌无力(myasthenia gravis,MG)合并胸腺瘤患者的临床病理特点及合理的外科治疗方案。方法回顾性分析47例MG合并胸腺瘤患者的临床资料。其胸腺瘤Masaoka病理分型Ⅰ期22例,Ⅱ期12例,Ⅲ期13例;MG改良Osserman法分型Ⅰ型20例,Ⅱa型14例,Ⅱb型7例,Ⅲ型6型。采用胸骨正中切口23例,前胸切口19例,后外侧切口5例。以累积生存率、MG转归为指标进行统计分析。结果本组平均随诊76.5(10~178)月。①11例(显性MG10例,隐性MG1例)术后围手术期出现肌无力危象(23.4%)。全组随诊共6例死于MG(12.8%),7例死于胸腺瘤(14.9%)。②显性MG32例,术后随诊5年总完全缓解率达34.4%,总改善率为62.5%。隐性MG15例,发作时间平均为术后37.7(0~137)d,出现MG后的5年自然缓解率为25.0%。③影响MG转归的因素为术后围手术期是否出现危象,出现者MG转归较差(P<0.05)。④经COX模型预后多因素分析,随诊中MG病情反复且有加重(优势比OR=2.16),胸腺瘤病理分期(OR=2.03)和手术方式(OR=1.63)为影响预后的主要因素。结论扩大胸腺切除术、术后肿瘤综合治疗、MG的围手术期综合处理及其长期治疗是提高疗效的有效手段。
Objective To investigate the clinicopathological features and reasonable surgical treatment of myasthenia gravis (MG) patients with thymoma. Methods The clinical data of 47 patients with MG thymoma were retrospectively analyzed. The thymoma Masaoka pathological type Ⅰ stage in 22 cases, 12 cases in stage Ⅱ, 13 cases in stage Ⅲ; MG modified Osserman method type Ⅰ 20 cases, Ⅱ a 14 cases, Ⅱ b 7 cases, Ⅲ 6 type. Twenty-three cases had a median incision of the sternum, 19 cases of anterior chest incision and 5 cases of posterior lateral incision. The cumulative survival rate, MG outcome as a statistical analysis. Results The average follow-up of 76.5 (10 ~ 178) months. ①11 cases (dominant MG10 cases, hidden MG1 cases) after perioperative myasthenia gravis crisis (23.4%). A total of 6 patients died of MG (12.8%) and 7 died of thymoma (14.9%). ② dominant MG32 cases, 5 years follow-up of the total complete remission rate was 34.4%, the overall improvement rate was 62.5%. 15 cases of latent MG, the average onset time was 37.7 (0 ~ 137) d after 5 months of MG natural remission rate was 25.0%. (3) The factors influencing the outcome of MG were whether there was a crisis in perioperative period after operation, and those who appeared were worse than those in MG (P <0.05). ④Multivariate analysis of the prognosis of COX model showed that MG disease was repeatedly and aggravate (odds ratio OR = 2.16), thymoma stage (OR = 2.03) and operation mode (OR = 1.63) as the main prognostic factors. Conclusion Expanding thymectomy, postoperative tumor comprehensive treatment, comprehensive treatment of perioperative MG and its long-term treatment are effective measures to improve the curative effect.