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与重症肌无力相关的胸腺瘤:对手术患者的长期观察研究

Thymic tumours associated with Myasthenia gravis: a long term observation study of operated patients.

作者信息

Bak V, Spalek P, Petrovajova T, Vichova B, Oravsky M, Luha J, Schnorrer M

机构信息

3rd Clinic of Surgery, University Hospital, Bratislava, Slovakia.

出版信息

Bratisl Lek Listy. 2013;114(8):464-8. doi: 10.4149/bll_2013_097.

Abstract

Primary tumours of thymus, thymoma and thymic carcinoma, are very rare, they represent less than 1 % of all neoplasms. The most common manifestation of thymoma with appearance of 40 to 50 % of the neuromuscular autoimmune disease is myasthenia gravis (MG). The performance of a complete resection is the most predictive indicator for long-term survival of patients with thymic tumour, also important prognostic factors are the histological type of thymic tumour and its invasiveness. The aim of our study is to study the long-term survival of patients after the resection of the tumour, as well as enhanced efficiency of radical thymectomy in the complex treatment of myasthenia gravis. From 1989 to 2010 we operated on 369 patients with MG. Out of 49 patients (13.7 %) 38 cases (76.6 %) were thymomas and 11 cases (23.4 %) thymic carcinomas. Complete removal of tumours (stage I, II, and III) were performed in 45 (92 %) cases. Of the 41 living patients (83.7 %), three (7.3 %) were diagnosed with metastatic thymic carcinoma or thymoma, and treated with adjuvant therapy. In one case, the patient died due to generalization of the thymic carcinoma. Statistical analysis with Kaplan-Meier method showed better overall survival of patients with thymoma than patients with thymic carcinoma. The difference in survival curves was not significant. (Mantel-Cox p = 0.479, Generalized Wilcoxon p = 0.326). In terms of treatment of Myasthenia gravis associated with thymoma, we achieved 70 % successful clinical and pharmacological remission. On the other hand, paraneoplastic MGAT has the worst prognosis of all the other forms of MG. The difference between MGAT to every other form of MG was statistically significant (Tab. 6, Fig. 1,Ref. 28).

摘要

胸腺原发性肿瘤,即胸腺瘤和胸腺癌,非常罕见,占所有肿瘤的比例不到1%。胸腺瘤最常见的表现(出现于40%至50%的病例中)是神经肌肉自身免疫性疾病,即重症肌无力(MG)。完整切除是胸腺肿瘤患者长期生存的最具预测性的指标,胸腺肿瘤的组织学类型及其侵袭性也是重要的预后因素。我们研究的目的是研究肿瘤切除术后患者的长期生存情况,以及在重症肌无力综合治疗中提高根治性胸腺切除术的效率。1989年至2010年,我们为369例重症肌无力患者实施了手术。在49例患者(13.7%)中,38例(76.6%)为胸腺瘤,11例(23.4%)为胸腺癌。45例(92%)患者的肿瘤(I期、II期和III期)被完整切除。在41例存活患者(83.7%)中,3例(7.3%)被诊断为转移性胸腺癌或胸腺瘤,并接受了辅助治疗。1例患者因胸腺癌全身转移死亡。采用Kaplan-Meier方法进行的统计分析显示,胸腺瘤患者的总体生存率优于胸腺癌患者。生存曲线的差异不显著(Mantel-Cox p = 0.479,广义Wilcoxon p = 0.326)。在与胸腺瘤相关的重症肌无力治疗方面,我们实现了70%的临床和药物成功缓解。另一方面,副肿瘤性MGAT在所有其他形式的MG中预后最差。MGAT与其他每种形式的MG之间的差异具有统计学意义(表6,图1,参考文献28)。

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