Ludwig Joseph A
Department of Sarcoma Medical Oncology, M.D. Anderson Cancer Center, Houston, Texas, USA.
Curr Opin Oncol. 2008 Jul;20(4):412-8. doi: 10.1097/CCO.0b013e328303ba1d.
As the second most common bone malignancy in children and adolescents, Ewing sarcoma family tumors represent almost 3% of pediatric cancers. Multidisciplinary management of patients with Ewing sarcoma has substantially improved the likelihood of survival for patients with localized disease. Yet, the prognosis of those with metastatic or recurrent disease has changed very little over the past three decades. Here, we focus on the current state-of-the-art in the diagnosis, staging, and treatment of Ewing's sarcoma and then highlight the most likely biological targets amenable to future therapies.
An improved understanding of the molecular biology of Ewing's sarcoma has led to clinical trials testing novel therapies specifically designed to thwart critical pathways responsible for this malignancy. Insulin-like growth factor-I receptor targeted monoclonal antibodies are just one example that has shown promise in early phase human clinical trials.
With an improved understanding of the genome, transcriptome, proteome, and other '-omic' events that promote and sustain Ewing pathogenesis, the use of nascent biologically targeted therapeutics is on the horizon. Understanding how and when to integrate such therapies into clinical practice, although challenging, may lead to a paradigm shift towards more personalized therapy.
尤因肉瘤家族性肿瘤是儿童和青少年中第二常见的骨恶性肿瘤，约占儿童癌症的3%。尤因肉瘤患者的多学科管理显著提高了局限性疾病患者的生存可能性。然而，在过去三十年中，转移性或复发性疾病患者的预后几乎没有变化。在此，我们聚焦于尤因肉瘤诊断、分期和治疗的当前前沿进展，然后突出最有可能适用于未来治疗的生物学靶点。
对尤因肉瘤分子生物学的深入理解促使开展了临床试验，测试专门设计用于阻断导致这种恶性肿瘤的关键通路的新型疗法。胰岛素样生长因子-I受体靶向单克隆抗体只是在早期人体临床试验中显示出前景的一个例子。
随着对促进和维持尤因肉瘤发病机制的基因组、转录组、蛋白质组及其他“组学”事件的理解不断加深，新型生物靶向疗法的应用即将出现。尽管具有挑战性，但了解如何以及何时将此类疗法整合到临床实践中，可能会导致向更个性化治疗的模式转变。
