重症肌无力伴胸腺瘤124例临床分析  

Clinical analysis of thymomas associated with myasthenia gravis:A report of 124 cases

作　　者：吴涛[1] 涂来慧[1] 王志农[2] 蒋建明[1] 张仁琴[1] 金海[2] 

机构地区：[1]第二军医大学长海医院神经内科,上海200433 [2]长海医院胸心外科

出　　处：《第二军医大学学报》2003年第11期1163-1166,共4页Academic Journal of Second Military Medical University

基　　金：上海市曙光计划基金(02SG30);上海市青年科技启明星计划(00QB14503)

摘　　要：目的:研究重症肌无力(myasthenia gravis,MG)伴胸腺瘤患者的临床特点、早期诊断和治疗与预后。方法:回顾分析124例经手术病理证实的伴胸腺瘤MG患者的病理和临床特征,评价胸部CT检查和骨骼肌柠檬酸提取物抗体(CAE-Ab)检测的诊断价值,分析MG治疗方法及远期疗效和预后。结果:124例MG按改良Osserman分型Ⅰ型有25例(20.2％),为最多;CEA-Ab阳性率为75.0％(66/88);胸部CT扫描显示纵隔占位性病变为88.7％(86/97)。胸腺瘤以淋巴细胞为主型最多,为55例(44.4％)。浸润程度按Masaoka分期标准分为Ⅰ期63例,Ⅱ期29例,Ⅲ期27例,Ⅳ期5例。92例Masaoka分期Ⅰ、Ⅱ期和22例Ⅲ期患者的肿瘤完全切除,并清除胸腺和周围脂肪组织,3例Ⅲ期和5例Ⅳ期部分切除,2例Ⅲ期仅做活检。79例患者给予皮质类固醇激素治疗,59例采用胸腺区放射治疗,5例难治性MG危象采用环磷酰胺治疗,4例转移性或复发性胸腺瘤采用联合化疗。随访1-16年,总有效率为72.6％。Masaoka分期Ⅰ期的5年和10年生存率分别为91.2％和76.9％,Ⅱ期的5年和10年生存率分别为86.7％和71.4％,明显高于Ⅲ期和Ⅳ期的35.7％和0(P<0.01)。结论:伴胸腺瘤MG以全身型为主,病理类型以淋巴细胞为主型常见,胸部CT扫描结合血清CAE-Ab检查有助于早期发现胸腺瘤。手术并不能消除MG症状。Objective:To study the early diagnosis,treatment,prognosis and clinical features of myasthenia gravis (MG) accompanied by thymomas. Methods: The clinical features of 124 cases surgically proved MG accompanied by thymomas were studied, the results of chest CT and citrite acid extraction (CAE) antibody were evaluated, and the treatment results and prognosis were analyzed. Results: Modified Osserman's classification showed type I patients were the most (25, 20. 2%); 75. 0% patients were CAE-Ab positive and 88. 7% chest CT scan showed thymomas; lymphocytic thymomas was the predominant (55,44. 4%). Of 124 patients, 63 had Masaoka's stage I thymomas, 29 stage I , 27 stage I and 5 stage IV. Masaoka's stage Ⅰ and Ⅱ and 22 stageⅢ had total excision, 3 stage I and 5 stage IV underwent partial resection and 2 stage I only had biopsy. Seventy-nine patients received corticosteroids therapy and 59 patients received radiotherapy. Five patients received cyclophosphamide due to refractory myasthenic crisis, 4 patients received combined chemotherapy due to metastasis or recurrence of thymomas. All patients were followed-up for 1-16 years. The total effective rate was 72. 6%. The 5-year and 10-year survival rates of Masaoka's stage 1 were 91. 2% and 76. 9% , of stage I were 86. 7% and 71. 4% , and of stage I and IV were 35. 7% and 0 respectively. Conclusion: Generalized MG is the major type in MG patients accompanied by thymomas, and the major pathological type is lymphocytic. Chest CT scan combined with serum CAE-Ab detection can increase the accuracy of thymoma early diagnosis. Corticosteroids therapy and radiotherapy should be based on the symptom of MG and pathology of thymomas.

关 键 词：重症肌无力 胸腺瘤 并发症 临床特点 诊断 治疗 

分 类 号：R746.1[医药卫生—神经病学与精神病学] R736.3[医药卫生—临床医学]