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重症肌无力伴侵袭性胸腺瘤的临床分析

[Clinical analysis of myasthenia gravis accompanied by invasive thymoma].

作者信息

Wakata N, Kawamura Y, Shin K, Kobayashi T, Kinoshita M

机构信息

Fourth Department of Internal Medicine, Toho University.

出版信息

Rinsho Shinkeigaku. 1991 Mar;31(3):260-5.

PMID:1893663
Abstract

Over the last 20 years, 6 patients with myasthenia gravis and invasive thymoma have been seen in our Department. These patients underwent non-total excision cases. This represents 2.5 percent of 242 myasthenia gravis patients in our series. We can see 17 such patients in the literature in Japan including our cases. The age ranged 20 to 77 years and the ratio of male to female was 10:7. Post-operative therapeutic methods for invasive thymoma included irradiation, steroid therapy and combination chemotherapy. Every method showed good therapeutic results, but steroid therapy and chemotherapy showed especially good therapeutic effects in the early stage of the disease. Though these therapeutic effects were better than those obtained in other malignant tumors, thymomas tended to reappear within several years, when tumors showed no response to any therapeutic method. It is well known that steroid therapy reduces the level of antiacetylcholine receptor antibody, whereas chemotherapy also reduces antiacetylcholine receptor antibody. Over all 5-year survival rate was 53%, and the 10-year survival rate was 29%. Three out of 6 cases of death were due to myasthenic crisis (50%) and 2 out of 6 cases were due to invasive tumor itself (38%). These results suggested that total excision of the thymoma, if possible, and for the remaining tumor, high doses of adrenocorticosteroids and combination chemotherapy seem to be treatments of choice.

摘要

在过去20年里,我们科室共收治了6例重症肌无力合并侵袭性胸腺瘤患者。这些患者均接受了非全切除手术。这占我们所治疗的242例重症肌无力患者的2.5%。在日本文献中,包括我们的病例在内,共可找到17例此类患者。患者年龄在20至77岁之间,男女比例为10:7。侵袭性胸腺瘤的术后治疗方法包括放疗、类固醇疗法和联合化疗。每种方法都显示出良好的治疗效果,但类固醇疗法和化疗在疾病早期显示出特别好的治疗效果。尽管这些治疗效果优于其他恶性肿瘤,但胸腺瘤在几年内往往会复发,此时肿瘤对任何治疗方法均无反应。众所周知,类固醇疗法可降低抗乙酰胆碱受体抗体水平,而化疗也可降低抗乙酰胆碱受体抗体水平。总体5年生存率为53%,10年生存率为29%。6例死亡病例中有3例死于重症肌无力危象(50%),6例中有2例死于侵袭性肿瘤本身(38%)。这些结果表明,如果可能,应尽量完整切除胸腺瘤,对于残留肿瘤,高剂量肾上腺皮质类固醇和联合化疗似乎是首选治疗方法。

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1
[Clinical analysis of myasthenia gravis accompanied by invasive thymoma].重症肌无力伴侵袭性胸腺瘤的临床分析
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2
[Management of myasthenia gravis in association with thymoma].[合并胸腺瘤的重症肌无力的管理]
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Kyobu Geka. 1993 Jan;46(1):61-8.
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[Myasthenia gravis and thymoma].重症肌无力与胸腺瘤
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Postthymectomy myasthenia gravis: relationship with thymoma and antiacetylcholine receptor antibody.胸腺切除术后重症肌无力:与胸腺瘤及抗乙酰胆碱受体抗体的关系
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Eur J Cardiothorac Surg. 2009 May;35(5):812-6; discussion 816. doi: 10.1016/j.ejcts.2009.01.014. Epub 2009 Feb 23.
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[Surgical treatment of myasthenia gravis in patients with thymoma].胸腺瘤患者重症肌无力的外科治疗
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[Management of myasthenia gravis (MG) associated with thymoma].[胸腺瘤相关重症肌无力(MG)的管理]
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