Pediatric Neuro-Oncology Unit, Pediatric Hematology Oncology Department, Hospital Infantil Universitario Niño Jesús, Avda. Menendez Pelayo 65, Madrid, 28009, Spain.
Department of Paediatric Haematology and Oncology, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic.
Childs Nerv Syst. 2024 Oct;40(10):3229-3239. doi: 10.1007/s00381-024-06458-w. Epub 2024 May 31.
Pediatric low-grade gliomas (PLGG) are commonly treated with a combination of surgery, radiotherapy, and chemotherapy. Recent trends prioritize reducing long-term morbidities, particularly in younger patients. While historically chemotherapy was reserved for cases progressing after radiotherapy, evolving recommendations now advocate for its early use, particularly in younger age groups. The carboplatin and vincristine (CV) combination stands as a standard systemic therapy for PLGG, varying in dosage and administration between North America and Europe. Clinical trials have shown promising response rates, albeit with varying toxicity profiles. Vinblastine has emerged as another effective regimen with minimal toxicity. TPCV, a regimen combining thioguanine, procarbazine, lomustine, and vincristine, was compared to CV in a Children's Oncology Group trial, showing comparable outcomes, but more toxicity. Vinorelbine, temozolomide, and metronomic chemotherapy have also been explored, with varied success rates and toxicity profiles. Around 40-50% of PLGG patients require subsequent chemotherapy lines. Studies have shown varied efficacy in subsequent lines, with NF1 patients generally exhibiting better outcomes. The identification of molecular drivers like BRAF mutations has led to targeted therapies' development, showing promise in specific molecular subgroups. Trials comparing targeted therapy to conventional chemotherapy aim to delineate optimal treatment strategies based on molecular profiles. The landscape of chemotherapy in PLGG is evolving, with a growing focus on molecular subtyping and targeted therapies. Understanding the role of chemotherapy in conjunction with novel treatments is crucial for optimizing outcomes in pediatric patients with low-grade gliomas.
小儿低度恶性胶质瘤 (PLGG) 通常采用手术、放疗和化疗相结合的方法进行治疗。近期的趋势侧重于减少长期的发病率，尤其是在年轻患者中。虽然历史上化疗仅用于放疗后进展的病例，但目前的推荐意见更倾向于早期使用，特别是在年龄较小的患者中。卡铂和长春新碱 (CV) 联合治疗是 PLGG 的标准全身治疗方法，在北美和欧洲的剂量和给药方式有所不同。临床试验显示出有希望的反应率，但毒性谱不同。长春碱已成为另一种具有最小毒性的有效方案。替莫唑胺联合卡铂和长春新碱（TPCV）方案在儿童肿瘤学组的一项试验中与 CV 进行了比较，结果显示两者的结局相似，但毒性更大。长春瑞滨、替莫唑胺和节拍化疗也进行了探索，其有效率和毒性谱各不相同。约 40-50%的 PLGG 患者需要后续化疗。研究表明，后续化疗的疗效存在差异，NF1 患者的结局通常更好。BRAF 突变等分子驱动因素的鉴定导致了靶向治疗的发展，在特定的分子亚组中显示出了前景。比较靶向治疗与常规化疗的试验旨在根据分子谱确定最佳治疗策略。PLGG 中的化疗格局正在不断发展，越来越注重分子分型和靶向治疗。了解化疗在与新型治疗联合应用中的作用对于优化小儿低度恶性胶质瘤患者的治疗结果至关重要。
