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胸腺瘤相关副肿瘤综合征的系统评价:切除病例的治疗方式、复发和结局。

A systematic review of paraneoplastic syndromes associated with thymoma: Treatment modalities, recurrence, and outcomes in resected cases.

机构信息

Keck School of Medicine, University of Southern California, Los Angeles, Calif.

Ohio University Heritage College of Osteopathic Medicine, Athens, Ohio.

出版信息

J Thorac Cardiovasc Surg. 2020 Jul;160(1):306-314.e14. doi: 10.1016/j.jtcvs.2019.11.052. Epub 2019 Dec 2.

DOI:10.1016/j.jtcvs.2019.11.052
PMID:31982129
Abstract

OBJECTIVE

Paraneoplastic syndromes associated with thymomas remain incompletely understood. The objective was to examine the association between surgically resected thymomas and paraneoplastic syndromes over the past half century.

METHODS

A primary PubMed/MEDLINE search was used to identify published articles describing paraneoplastic syndromes associated with thymomas from 1960 to 2019. A secondary search identified additional articles referenced in the articles found in the primary search. Kaplan-Meier and log-rank test were used for time-to-event data analyses.

RESULTS

From 407 articles describing 507 patients, 123 different paraneoplastic syndromes were associated with thymoma. The 5 most common paraneoplastic syndromes were myasthenia gravis, pure red cell aplasia, lichen planus, Good syndrome, and limbic encephalitis. Complete or partial resolution of paraneoplastic syndrome symptoms after surgery was noted in 76% of patients, of whom 21% had a relapse or new paraneoplastic syndrome onset after surgery. The most common adjunctive therapy associated with resolution of paraneoplastic syndrome was corticosteroids (30%). For all patients after surgery, thymoma recurrence was observed in 17% of cases, whereas recurrence of paraneoplastic syndrome was observed in 34% of cases, and both were observed in approximately 11% of cases. The 5- and 10-year overall survivals were 78% and 66%, respectively. Improved overall survival was associated with patients who had total resolution from paraneoplastic syndrome.

CONCLUSIONS

A comprehensive assessment of publications over the past half century suggests that a multimodal treatment approach that includes surgical resection of thymomas is able to achieve paraneoplastic syndrome resolution in a majority of patients. Onset of new paraneoplastic syndromes after surgery is associated with the recurrence of the first paraneoplastic syndrome, and resolution of paraneoplastic syndrome is associated with improved overall survival.

摘要

目的

与胸腺瘤相关的副肿瘤综合征仍不完全了解。本研究旨在检查过去半个世纪中手术切除的胸腺瘤与副肿瘤综合征之间的关联。

方法

通过初级 PubMed/MEDLINE 搜索,确定了 1960 年至 2019 年描述胸腺瘤相关副肿瘤综合征的已发表文章。二次搜索确定了在初级搜索中找到的文章中引用的其他文章。使用 Kaplan-Meier 和对数秩检验进行生存数据分析。

结果

从描述 507 例患者的 407 篇文章中,确定了 123 种不同的副肿瘤综合征与胸腺瘤相关。最常见的 5 种副肿瘤综合征为重症肌无力、纯红细胞再生障碍、扁平苔藓、Good 综合征和边缘性脑炎。76%的患者术后副肿瘤综合征症状完全或部分缓解,其中 21%的患者术后复发或出现新的副肿瘤综合征。与缓解副肿瘤综合征相关的最常见辅助治疗是皮质类固醇(30%)。对于所有手术后的患者,胸腺瘤复发率为 17%,而副肿瘤综合征复发率为 34%,两者的发生率均约为 11%。5 年和 10 年的总生存率分别为 78%和 66%。总体生存的改善与副肿瘤综合征完全缓解的患者相关。

结论

对过去半个世纪的文献进行综合评估表明,包括胸腺瘤切除术在内的多模式治疗方法能够使大多数患者的副肿瘤综合征得到缓解。术后新发副肿瘤综合征与首发副肿瘤综合征的复发相关,而副肿瘤综合征的缓解与总体生存的改善相关。

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