Jujui-Eam Apisak, Sirachainan Nongnuch, Hongeng Suradej, Hansasuta Ake, Boongird Atthaporn, Tritanon Oranan, Dhanachai Mantana, Swangsilpa Thiti, Ruangkanchanasetr Rawee, Worawongsakul Rasin, Puataweepong Putipun
Radiation Oncology Unit, Department of Diagnostic and Therapeutic Radiology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Ratchathewi, 10400, Bangkok, Thailand.
Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Childs Nerv Syst. 2023 May;39(5):1173-1182. doi: 10.1007/s00381-022-05809-9. Epub 2022 Dec 27.
A multimodality approach is generally considered for pediatric low-grade gliomas (LGG); however, the optimal management remains uncertain. The objective of the study was to evaluate treatment outcomes of pediatric LGG, focusing on long-term survival and factors related to outcomes.
A retrospective review of 77 pediatric LGG cases treated at Ramathibodi Hospital, Thailand between 2000 and 2018 was performed. The inclusion criteria were all pediatric LGG cases aged ≤ 15 years. Diffuse intrinsic pontine gliomas and spinal cord tumors were excluded.
The median follow-up time was 8.2 years (range, 0.6-19.7). The median age at diagnosis was 6.2 years (interquartile range, 3.6-11.4). Treatments modality included tumor surgery (93%), chemotherapy (40%), and radiation therapy (14%). The 10-year overall survival (OS) and 10-year progression-free survival were 94% and 59%, respectively, for the entire cohort. The 10-year OS was 100% in three subgroups of patients: pilocytic subtype, WHO grade 1 tumors, and recipient of gross total resection. After multivariable analysis, no tumor surgery had a significantly unfavorable influence on overall survival.
With a multimodality approach, pediatric LGGs had excellent outcome. Gross total resection is the standard primary treatment. Chemotherapy is the alternative standard treatment in incomplete resection cases, unresectable patients, or patients with progressive disease. Radiation therapy should be reserved as a salvage treatment option because of late complications that usually affect patients' quality of life.
小儿低级别胶质瘤（LGG）通常采用多模态治疗方法；然而，最佳治疗方案仍不明确。本研究的目的是评估小儿LGG的治疗效果，重点关注长期生存率及与预后相关的因素。
对2000年至2018年期间在泰国拉玛蒂博迪医院接受治疗的77例小儿LGG病例进行回顾性分析。纳入标准为所有年龄≤15岁的小儿LGG病例。排除弥漫性脑桥内在型胶质瘤和脊髓肿瘤。
中位随访时间为8.2年（范围0.6 - 19.7年）。诊断时的中位年龄为6.2岁（四分位间距3.6 - 11.4岁）。治疗方式包括肿瘤手术（93%）、化疗（40%）和放疗（14%）。整个队列的10年总生存率（OS）和10年无进展生存率分别为94%和59%。在三个亚组患者中10年OS为100%：毛细胞型亚型、世界卫生组织1级肿瘤以及接受全切除的患者。多变量分析后，未进行肿瘤手术对总生存无显著不利影响。
采用多模态治疗方法，小儿LGG预后良好。全切除是标准的主要治疗方法。化疗是不完全切除病例、不可切除患者或疾病进展患者的替代标准治疗方法。由于放疗的晚期并发症通常会影响患者生活质量，应将其作为挽救性治疗选择。
