*Molecular Pathology Program, Institute of Biomedical Research of Salamanca-Centro de Investigación del Cáncer, Centro de Investigación del Cancér (IBSAL-CIC), Campus Miguel de Unamuno S/N, Salamanca †Pathology Department, Hospital Universitario Virgen del Rocio-IBiS, Sevilla, Spain.
Adv Anat Pathol. 2014 Jan;21(1):44-62. doi: 10.1097/PAP.0000000000000003.
Ewing Sarcoma is a developmental tumor characterized by balanced chromosomal translocations and formation of new fusion genes, which are the main hallmark of this rare entity. Despite the vast knowledge regarding the molecular aspects of this rare malignancy obtained in the last few years, including the discovery of new therapeutic targets, many questions still remain open. In this review we focus on the research on targeted therapies in this malignancy, and discussed some bottlenecks related to this such as the possible role of pathologists, the availability of samples, the lack of appropriate animal models, and the resources needed to carry out preclinical and clinical research.
尤因肉瘤是一种发育性肿瘤,其特征为染色体平衡易位和新融合基因的形成,这是该罕见实体的主要标志。尽管近年来在分子方面对这种罕见恶性肿瘤有了广泛的了解,包括发现新的治疗靶点,但仍有许多问题悬而未决。在这篇综述中,我们重点介绍了针对这种恶性肿瘤的靶向治疗研究,并讨论了一些与之相关的瓶颈问题,如病理学家的可能作用、样本的可用性、缺乏合适的动物模型以及开展临床前和临床研究所需的资源。