Division of Pediatric Hematology/Oncology, Department of Pediatrics and Adolescent Medicine, Mayo Clinic, 200 First St SW, Rochester, MN, 55905, USA,
Curr Oncol Rep. 2014;16(8):398. doi: 10.1007/s11912-014-0398-9.
Low-grade gliomas (LGGs) represent the most common childhood brain tumors and are a histologically heterogenous group of tumors. Most LGGs are surgically resectable with excellent 10-year overall survival outcomes of more than 90 % with surgery alone. Tumors not amenable to surgical resection and those with an aggressive biology are more challenging to treat. Conventional radiotherapy is a more efficacious method of long-term tumor control than chemotherapy. However, radiation is associated with significant cognitive, endocrine, and cerebrovascular late effects, making chemotherapy an often-preferred modality over radiotherapy, especially in younger children. Multiple chemotherapy regimens have been evaluated over the past few decades with comparable survival outcomes and differing toxicity profiles. Newer regimens containing antiangiogenic agents also show promise. Recent molecular studies have implicated the BRAF oncogene, a key regulator of the MAPK pathway, and the AKT/mTOR pathway in pediatric LGG tumorigenesis. This has opened up promising new avenues for targeted therapy, with many agents currently under investigation.
低级别胶质瘤 (LGG) 是儿童中最常见的脑肿瘤，也是一组组织学异质性的肿瘤。大多数 LGG 可通过手术切除，单独手术的 10 年总生存率超过 90%。对于那些无法手术切除的肿瘤和具有侵袭性生物学特性的肿瘤，治疗起来更为困难。与化疗相比，常规放疗是一种更有效的长期肿瘤控制方法。然而，放疗会导致明显的认知、内分泌和脑血管的迟发性并发症，使得化疗成为比放疗更受欢迎的治疗方法，尤其是在年幼的儿童中。在过去几十年中，已经评估了多种化疗方案，它们具有相似的生存结果和不同的毒性特征。含有抗血管生成药物的新方案也显示出前景。最近的分子研究表明，BRAF 癌基因（MAPK 通路的关键调节因子）和 AKT/mTOR 通路参与了儿童 LGG 的肿瘤发生。这为靶向治疗开辟了有希望的新途径，目前有许多药物正在研究中。
