Suppr超能文献

[罕见病研究与诊断中的挑战]

[Challenges in the research and diagnosis of rare diseases].

作者信息

Marx Alexander, Ströbel Philipp, Bremmer Felix, Nettersheim Daniel, Rupp Niels J, Wardelmann Eva, Hartmann Wolfgang

机构信息

Institut für Pathologie, Universitätsmedizin Göttingen, Georg-August-Universität Göttingen, Robert-Koch-Straße 40, 37075, Göttingen, Deutschland.

Klinik für Urologie, Urologisches Forschungslabor, Translationale Uro-Onkologie, Medizinische Fakultät und Universitätsklinikum Düsseldorf, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Deutschland.

出版信息

Pathologie (Heidelb). 2025 May;46(3):142-151. doi: 10.1007/s00292-025-01426-w. Epub 2025 Apr 3.

Abstract

Molecular methods have improved the diagnosis of many rare tumors but have also revealed their limitations. Gene fusions that originally appeared specific occur "promiscuously" in biologically distinct mesenchymal and epithelial tumors, underscoring the importance of integrated morphologic-molecular diagnostics. By contrast, similar tumor biology-which is difficult to prove in rare tumors-supports the concept of entity-defining gene fusions for a spectrum of morphologically diverse tumors. Still other rare tumors have no diagnostically or prognostically helpful molecular profile, and their rarity and lack of authentic tumor models are obstacles to the use of, for example, new single-cell-based molecular or AI-assisted morphological methods and preclinical functional analyses. These peculiarities of rare tumors are illustrated by thymic, testicular, salivary gland, and soft tissue neoplasms.

摘要

分子方法改善了许多罕见肿瘤的诊断,但也揭示了其局限性。最初看似具有特异性的基因融合在生物学上不同的间充质和上皮肿瘤中“随意”出现,凸显了综合形态学-分子诊断的重要性。相比之下,相似的肿瘤生物学——这在罕见肿瘤中难以证实——支持了为一系列形态多样的肿瘤定义实体的基因融合概念。还有其他一些罕见肿瘤没有诊断或预后方面有用的分子特征,它们的罕见性以及缺乏真实的肿瘤模型是使用例如新的基于单细胞的分子或人工智能辅助形态学方法以及临床前功能分析的障碍。胸腺、睾丸、唾液腺和软组织肿瘤体现了罕见肿瘤的这些特性。

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验