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伴有胸腺上皮肿瘤的重症肌无力:日本数据库的回顾性分析

Myasthenia gravis with thymic epithelial tumour: a retrospective analysis of a Japanese database.

作者信息

Nakajima Jun, Okumura Meinoshin, Yano Motoki, Date Hiroshi, Onuki Takuya, Haniuda Masayuki, Sano Yoshifumi

机构信息

Department of Thoracic Surgery, The University of Tokyo Graduate School of Medicine, Tokyo, Japan

Department of General Thoracic Surgery, Osaka University, Osaka, Japan.

出版信息

Eur J Cardiothorac Surg. 2016 May;49(5):1510-5. doi: 10.1093/ejcts/ezv380. Epub 2015 Nov 3.

Abstract

OBJECTIVES

Myasthenia gravis (MG) has been reported to correlate with earlier stage and Type B thymomas by the World Health Organization classification. We analysed a large database of clinical characteristics of patients with MG and thymic epithelial tumours to elucidate whether the severity of MG affected postoperative survival of those with thymic epithelial neoplasms.

METHODS

We conducted a multi-institutional study on the patients who had undergone surgical treatment for thymic epithelial tumours between 1991 and 2010. We examined Masaoka stage, pathological type, serum titre of antiacetylcholine receptor antigen, severity of MG and postoperative prognosis of the patients with or without MG.

RESULTS

Of the 2835 registered patients at 32 institutes belonging to the Japanese Association for Research on the Thymus, 2638 were eligible for the study. MG was present in 598 patients (23%). Patients with MG had thymic epithelial tumours with significantly earlier stage (P = 0.0082) and significantly smaller tumours (P = 0.000) than those without. Type A, Type AB thymomas and thymic carcinomas were less frequently observed in patients with MG. Three of 304 patients (1%) with thymic carcinomas had MG preoperatively. Serum titres of antiacetylcholine receptor antibodies were positive in 98% of patients with MG, and 23% of those without. Patients with generalized MG had significantly higher titres of antiacetylcholine receptor antibodies than those with ocular MG (P = 0.000). The postoperative 30-day mortality rate was 0.3%. Postoperative 5- and 10-year survival rates of thymoma patients with MG and those without MG were 94 and 96, and 89 and 89%, respectively. We found no statistical difference in the postoperative survival rate or recurrence-free rate between the two groups. We found no significant statistical differences of these rates by MGFA classification or surgical approach.

CONCLUSIONS

We conclude that earlier stage, smaller size or Type B thymomas are more frequently associated with MG, and MG may have no impact on the overall survival of patients with thymoma. We suggest that postoperative survival time of the patients with MG and thymic epithelial tumours may be mainly affected by the tumours, not by MG.

摘要

目的

据报道,重症肌无力(MG)与世界卫生组织分类中的早期及B型胸腺瘤相关。我们分析了一个关于MG患者和胸腺上皮肿瘤患者临床特征的大型数据库,以阐明MG的严重程度是否会影响胸腺上皮肿瘤患者的术后生存率。

方法

我们对1991年至2010年间接受胸腺上皮肿瘤手术治疗的患者进行了一项多机构研究。我们检查了有或无MG患者的Masaoka分期、病理类型、抗乙酰胆碱受体抗原的血清滴度、MG的严重程度及术后预后。

结果

在日本胸腺研究协会所属32家机构登记的2835例患者中,2638例符合研究条件。598例患者(23%)患有MG。与无MG患者相比,患有MG的患者胸腺上皮肿瘤分期明显更早(P = 0.0082),肿瘤明显更小(P = 0.000)。A 型、AB型胸腺瘤和胸腺癌在MG患者中较少见。304例胸腺癌患者中有3例(1%)术前患有MG。98%的MG患者抗乙酰胆碱受体抗体血清滴度呈阳性,无MG患者中这一比例为23%。全身型MG患者的抗乙酰胆碱受体抗体滴度显著高于眼肌型MG患者(P = 0.000)。术后30天死亡率为0.3%。伴有MG和不伴有MG的胸腺瘤患者术后5年和10年生存率分别为94%和96%,以及89%和89%。我们发现两组之间的术后生存率或无复发生存率无统计学差异。根据MGFA分类或手术方式,这些比率也无显著统计学差异。

结论

我们得出结论,早期、较小尺寸或B型胸腺瘤更常与MG相关,且MG可能对胸腺瘤患者的总体生存无影响。我们认为,MG患者和胸腺上皮肿瘤患者的术后生存时间可能主要受肿瘤影响,而非MG。

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