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胸腺瘤与重症肌无力的关联:手术治疗的肿瘤学及神经学结果

Association of thymoma and myasthenia gravis: oncological and neurological results of the surgical treatment.

作者信息

Lucchi Marco, Ricciardi Roberta, Melfi Franca, Duranti Leonardo, Basolo Fulvio, Palmiero Gerardo, Murri Luigi, Mussi Alfredo

机构信息

Cardiac and Thoracic Department, Division of Thoracic Surgery, University of Pisa, Pisa, Italy.

出版信息

Eur J Cardiothorac Surg. 2009 May;35(5):812-6; discussion 816. doi: 10.1016/j.ejcts.2009.01.014. Epub 2009 Feb 23.

DOI:10.1016/j.ejcts.2009.01.014
PMID:19237289
Abstract

BACKGROUND

Thymoma occurs in about 10-20% of myasthenic patients and in turn, 20-25% of patients with a thymoma have myasthenia gravis. Both diseases are treated by surgery. The aims of this study were to analyze the clinical features of these patients and the oncological and neurological outcomes after thymectomy.

METHODS

Clinical and pathological data, complete remission rate as well as overall survival rates were retrospectively analyzed in a cohort of myasthenic patients who underwent extended thymectomy for thymoma between 1993 and 2006.

RESULTS

One hundred and twenty-three patients (60 m and 63 f) with a mean age of 56 years (range 22-83) underwent extended thymectomy. The WHO histological classification was: A in 22 cases, AB in 18, B1 in 33, B2 in 22, and B3 in 28. The Masaoka clinical staging was: I in 10 cases, IIA in 33, IIB in 50, III in 14, IVA in 15, IVB in 1. We experienced 2 postoperative deaths. With a overall mean follow-up of 76 months 42 patients had a complete remission, 39 a remission with medications, 35 an improvement of the symptoms, 3 remained nearly in the same status and 4 worsened. At the last follow-up, 112 patients were alive; 11 with disease. Four deaths were related to the tumor. Actuarial 5- and 10-year survival was 93.4% and 79.6%, respectively.

CONCLUSIONS

Neurological outcome of the extended thymectomy in myasthenic thymoma patients was satisfactory. Higher complete remission rate is expected in early stage thymoma. Regarding the overall survival it was dependent on the Masaoka stage, the WHO classification and the achievement of complete remission of myasthenic symptoms.

摘要

背景

胸腺瘤发生于约10% - 20%的重症肌无力患者中,反之,20% - 25%的胸腺瘤患者患有重症肌无力。这两种疾病均通过手术治疗。本研究的目的是分析这些患者的临床特征以及胸腺切除术后的肿瘤学和神经学结局。

方法

对1993年至2006年间因胸腺瘤接受扩大胸腺切除术的一组重症肌无力患者的临床和病理数据、完全缓解率以及总生存率进行回顾性分析。

结果

123例患者(60例男性和63例女性)接受了扩大胸腺切除术,平均年龄56岁(范围22 - 83岁)。世界卫生组织组织学分类为:A型22例,AB型18例,B1型33例,B2型22例,B3型28例。马萨oka临床分期为:I期10例,IIA期33例,IIB期50例,III期14例,IVA期15例,IVB期1例。我们经历了2例术后死亡。总体平均随访76个月,42例患者完全缓解,39例药物缓解,35例症状改善,3例几乎维持原状,4例病情恶化。在最后一次随访时,112例患者存活;11例有疾病。4例死亡与肿瘤相关。5年和10年精算生存率分别为93.4%和79.6%。

结论

重症肌无力胸腺瘤患者扩大胸腺切除术的神经学结局令人满意。早期胸腺瘤有望获得更高的完全缓解率。关于总生存率,它取决于马萨oka分期、世界卫生组织分类以及重症肌无力症状的完全缓解情况。

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