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胸腺上皮肿瘤的生物学谱:现状与未来展望。

The biologic spectrum of thymic epithelial neoplasms: current status and future prospects.

机构信息

Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, New York 14263, USA.

出版信息

J Natl Compr Canc Netw. 2010 Nov;8(11):1322-8. doi: 10.6004/jnccn.2010.0097.

Abstract

Thymoma is the most common anterior mediastinal tumor in adults and is frequently associated with autoimmune disorders such as myasthenia gravis. Thymomas are a diverse group of epithelial neoplasms with a behavioral spectrum that spans the complete clinical gamut from entirely benign to highly aggressive, lethal thymic carcinomas. The biologic behavior seems to depend primarily on the clinical stage at presentation and histologic subtype. This article discusses thymic organogenesis, Masaoka staging, WHO histologic classification of thymoma and thymic carcinoma, and selected molecular characteristics that highlight this diversity. This discussion will further underscore both the similarities and differences between categories of thymic epithelial neoplasms and offer support for the notion that tumor heterogeneity and/or tumor progression may explain the observed clinical variation in behavior. Recommendations are offered for future investigational approaches to further the understanding of the complexity of these tumors.

摘要

胸腺瘤是成年人最常见的前纵隔肿瘤,常与自身免疫性疾病如重症肌无力有关。胸腺瘤是一组多样化的上皮性肿瘤,其行为谱跨越了从完全良性到高度侵袭性、致命性胸腺癌的整个临床范围。其生物学行为似乎主要取决于发病时的临床分期和组织学亚型。本文讨论了胸腺器官发生、Masaoka 分期、胸腺瘤和胸腺癌的 WHO 组织学分类以及突出这种多样性的选定分子特征。这一讨论将进一步强调胸腺上皮性肿瘤类别的相似性和差异性,并支持肿瘤异质性和/或肿瘤进展可能解释观察到的临床行为变异的观点。为进一步了解这些肿瘤的复杂性,提出了未来研究方法的建议。

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