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小儿胸腺瘤:两例报告及文献综述

Pediatric thymomas: report of two cases and comprehensive review of the literature.

作者信息

Fonseca Annabelle L, Ozgediz Doruk E, Christison-Lagay Emily R, Detterbeck Frank C, Caty Michael G

机构信息

Department of Pediatric Surgery, Yale School of Medicine, FMB 107, 333 Cedar Street, New Haven, CT, 06511, USA,

出版信息

Pediatr Surg Int. 2014 Mar;30(3):275-86. doi: 10.1007/s00383-013-3438-x. Epub 2013 Dec 10.

Abstract

PURPOSE

Thymomas are rare pediatric malignancies with indolent behavior. There are fewer than 50 reported cases and no comprehensive review. We sought to evaluate our recent experience with pediatric thymomas, and comprehensively review the extant literature.

METHODS

A systematic search of the PubMed database was performed using keywords: "thymoma", "pediatric", "juvenile", "childhood", and "child". Additional studies were identified by a manual search of the reference list.

RESULTS

We report two patients with thymomas. We identified 22 case reports or series that described 48 patients; 62 % were male, 15 % presented with myasthenia gravis. Fifty percent were Masaoka Stage I, 15 % were Stage II, 13 % were Stage III, and 23 % were Stage IV. Four patients with early stage (I or II) disease were treated with adjuvant therapies in addition to surgical excision, while five patients with late stage (III or IV) disease treated with surgical excision alone. Of studies reporting at least 2-year follow-up, survival was 71 %.

CONCLUSION

Pediatric thymomas are rare tumors with a slight male predominance. Wide variations were observed in the treatment of thymomas across all stages. Our review indicates a need for large database and multi-institutional studies to clearly elucidate clinical course, prognostic factors and outcome.

摘要

目的

胸腺瘤是一种行为惰性的罕见儿科恶性肿瘤。报告的病例少于50例,且尚无全面综述。我们旨在评估我们近期治疗儿科胸腺瘤的经验,并全面回顾现有文献。

方法

使用关键词“胸腺瘤”“儿科”“青少年”“儿童期”和“儿童”对PubMed数据库进行系统检索。通过手动检索参考文献列表确定其他研究。

结果

我们报告了2例胸腺瘤患者。我们确定了22篇病例报告或系列研究,共描述了48例患者;其中62%为男性,15%伴有重症肌无力。50%为Masaoka I期,15%为II期,13%为III期,23%为IV期。4例早期(I或II期)疾病患者除手术切除外还接受了辅助治疗,而5例晚期(III或IV期)疾病患者仅接受了手术切除。在报告至少2年随访的研究中,生存率为71%。

结论

儿科胸腺瘤是罕见肿瘤,男性略占优势。各阶段胸腺瘤的治疗存在很大差异。我们的综述表明,需要大型数据库和多机构研究来明确阐明临床病程、预后因素和结局。

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