Burchill Susan Ann
Candlelighter's Children's Cancer Research Group, Cancer Research UK Clinical Centre, Leeds Institute of Molecular Medicine, St James's University Hospital, Leeds, UK.
Expert Rev Anticancer Ther. 2008 Oct;8(10):1675-87. doi: 10.1586/14737140.8.10.1675.
Ewing's sarcoma is one of the few solid tumors for which the underlying molecular genetic abnormality has been described: rearrangement of the EWS gene on chromosome 22q12 with an ETS gene family member. These translocations define the Ewing's sarcoma family of tumors (ESFT) and provide a valuable tool for their accurate and unequivocal diagnosis. They also represent ideal targets for the development of tumor-specific therapeutics. Although secondary abnormalities occur in over 80% of primary ESFT the clinical utility of these is currently unclear. However, abnormalities in genes that regulate the G(1)/S checkpoint are frequently described and may be important in predicting outcome and response. Increased understanding of the molecular events that arise in ESFT and their role in the development and maintenance of the malignant phenotype will inform the improved stratification of patients for therapy and identify targets and pathways for the design of more effective cancer therapeutics.
22号染色体q12区域的EWS基因与ETS基因家族成员发生重排。这些易位定义了尤因肉瘤肿瘤家族（ESFT），并为其准确无误的诊断提供了有价值的工具。它们也是开发肿瘤特异性疗法的理想靶点。尽管超过80%的原发性ESFT会出现继发性异常，但其临床效用目前尚不清楚。然而，经常有人描述调节G(1)/S 检查点的基因异常，这可能对预测预后和反应很重要。对ESFT中出现的分子事件及其在恶性表型的发生和维持中的作用有了更多了解，将有助于改进患者治疗分层，并确定设计更有效癌症疗法的靶点和途径。
