Division of Pediatric Hematology-Oncology, Hasbro Children's Hospital, The Warren Alpert Medical School of Brown University, Providence, Rhode Island, USA.
Division of Hematology-Oncology, Cancer and Blood Disease Institute, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Pediatr Blood Cancer. 2023 Sep;70 Suppl 6(Suppl 6):e30562. doi: 10.1002/pbc.30562. Epub 2023 Jul 14.
Extracranial germ cell tumors (GCT) are a biologically diverse group of tumors occurring in children, adolescents, and young adults. The majority of patients have excellent outcomes, but treatment-related toxicities impact their quality of survivorship. A subset of patients succumbs to the disease. Current unmet needs include clarifying which patients can be safely observed after initial surgical resection, refinement of risk stratification to reduce chemotherapy burden in patients with standard-risk disease, and intensify therapy for patients with poor-risk disease. Furthermore, enhancing strategies for detection of minimal residual disease and early detection of relapse, particularly in serum tumor marker-negative histologies, is critical. Improving the understanding of the developmental and molecular origins of GCTs may facilitate discovery of novel targets. Future efforts should be directed toward assessing novel therapies in a biology-driven, biomarker-defined, histology-specific, risk-stratified patient population. Fragmentation of care between subspecialists restricts the unified study of these rare tumors. It is imperative that trials be conducted in collaboration with national and international cooperative groups, with harmonized data and biospecimen collection. Key priorities for the Children's Oncology Group (COG) GCT Committee include (a) better understanding the biology of GCTs, with a focus on molecular targets and mechanisms of treatment resistance; (b) strategic development of pediatric and young adult clinical trials; (c) understanding late effects of therapy and identifying individuals most at risk; and (d) prioritizing diversity, equity, and inclusion to reduce cancer health disparities and studying the impacts of social determinants of health on outcomes.
颅外生殖细胞肿瘤 (GCT) 是一组生物学上多样化的肿瘤,发生在儿童、青少年和年轻成人中。大多数患者的预后良好,但治疗相关的毒性会影响其生存质量。一部分患者会死于该疾病。目前未满足的需求包括阐明哪些患者在初始手术切除后可以安全观察、细化风险分层以减少标准风险疾病患者的化疗负担,以及为预后不良的患者强化治疗。此外,增强检测微小残留病和早期检测复发的策略至关重要,尤其是在血清肿瘤标志物阴性组织学中。提高对 GCT 发育和分子起源的认识可能有助于发现新的靶点。未来的努力应致力于在生物学驱动、生物标志物定义、组织学特异性、风险分层的患者群体中评估新的治疗方法。亚专科医生之间的治疗分割限制了对这些罕见肿瘤的统一研究。必须与国家和国际合作团体合作进行试验,协调数据和生物样本收集。儿童肿瘤学组 (COG) GCT 委员会的重点包括:(a) 更好地了解 GCT 的生物学,重点是分子靶点和治疗耐药机制;(b) 制定儿科和青年临床试验的战略;(c) 了解治疗的晚期影响并确定风险最大的个体;(d) 优先考虑多样性、公平性和包容性,以减少癌症健康差距,并研究健康决定因素对结果的影响。