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尤因肉瘤
Ewing’s Sarcoma


Nicolò Riggi ... 肿瘤 • 2021.01.14

1920年,在纽约病理学会(New York Pathological Society)的一次会议上,詹姆斯·尤因(James Ewing)将一名14岁女童身上的罕见肿瘤描述为“弥漫性骨内皮瘤”1。该肿瘤最初被诊断为骨肉瘤,但其结构、细胞形态学特征及对放疗的高度敏感性使尤因认为这是一种独立疾病,甚至推测其起源于内皮细胞1。他后来还报告了其他青少年发生的类似肿瘤,病理科医师根据这些肿瘤共同的形态学和免疫组化特征,将其称为尤因肉瘤、Askin瘤和外周原始神经外胚层肿瘤。在确诊尤因肉瘤道路上,第一个里程碑式发现出现在70多年后,当时发现了可定义尤因肉瘤的最常见染色体易位2。在尤因做出上述开创性观察一个世纪后,以其名字命名的恶性肿瘤成为一次基因重排后即发展成实体瘤的范例。

在这篇综述中,我们讨论了尤因肉瘤的临床特征和发病机制,以及现有和实验性治疗方法。就机制角度而言,我们总结了独特染色体易位利用允许细胞(permissive cell)的表观遗传机制来改变其转录组,并产生甚至可逃避最高强度常规治疗的异质性癌症的机制。





作者信息

Nicolò Riggi, M.D., Ph.D., Mario L. Suvà, M.D., Ph.D., and Ivan Stamenkovic, M.D.
From the Institute of Pathology, Faculty of Biology and Medicine, University of Lausanne and Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland (N.R., I.S.); and the Department of Pathology and Center for Cancer Research, Massachusetts General Hospital and Harvard Medical School, Boston, and the Broad Institute of Harvard University and the Massachusetts Institute of Technology, Cambridge — both in Massachusetts (M.L.S.). Address reprint requests to Dr. Stamenkovic at Pathologie Expérimentale, Institut de Pathologie, 25 rue du Bugnon, CH-1011 Lausanne, Switzerland, or at ivan.stamenkovic@chuv.ch.

 

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