McCabe Martin G, Valteau-Couanet Dominique
Prog Tumor Res. 2016;43:74-86. doi: 10.1159/000447075. Epub 2016 Sep 5.
Embryonal tumors classically occur in young children, some principally within the first year of life. Prospective national and international clinical trials during recent decades have brought about progressive improvements in survival, and associated biological studies have advanced our understanding of tumor biology, in some cases allowing biological tumor characteristics to be harnessed for therapeutic benefit. Embryonal tumors continue to occur, albeit less commonly, during childhood, adolescence and throughout adulthood. These tumors are less well understood, usually not managed according to standardized protocols and rarely included in clinical trials. Survival outcomes are generally poorer than their childhood equivalents. We present here a summary of the published literature on embryonal tumors that present ectopically during adolescence and adulthood. We show that for some tumors protocol-driven treatment, supported by accurate and complete diagnostics and staging, can result in equivalent outcomes to those seen during childhood. We make the case that clinical trial eligibility criteria should be disease-based rather than age-based, and support improvements in dialogue between children's and adults' cancer clinicians to improve outcomes for these rare tumors.
胚胎性肿瘤典型地发生于幼儿，有些主要发生在生命的第一年。近几十年来，全国性和国际性的前瞻性临床试验使生存率逐步提高，相关的生物学研究也增进了我们对肿瘤生物学的理解，在某些情况下能够利用肿瘤的生物学特性实现治疗益处。胚胎性肿瘤在儿童期、青春期及整个成年期均会发生，尽管较为少见。这些肿瘤的情况鲜为人知，通常未按照标准化方案进行管理，也很少被纳入临床试验。其生存结果总体上比儿童期的同类肿瘤更差。我们在此总结已发表的关于青春期和成年期异位出现的胚胎性肿瘤的文献。我们表明，对于某些肿瘤，在准确完整的诊断和分期支持下，采用方案驱动的治疗可产生与儿童期所见相当的结果。我们认为临床试验的入选标准应以疾病为基础而非以年龄为基础，并支持改善儿童癌症临床医生和成人癌症临床医生之间的沟通，以改善这些罕见肿瘤的治疗结果。
