From: St Jude Children's Research Hospital, Memphis, TN.
J Clin Oncol. 2017 Jul 20;35(21):2364-2369. doi: 10.1200/JCO.2017.73.1265. Epub 2017 Jun 22.
Ependymoma is a locally aggressive tumor with metastatic potential that arises in diverse locations throughout the brain and spine in children. Tumor and treatment may result in significant morbidity. Cure remains elusive for many patients owing to diverse biology and resistance to conventional therapy. The implementation of systematic postoperative irradiation in clinical trials during the past 20 years has increased the proportion of patients achieving durable disease control with excellent results, as measured by objective functional outcome measures. Clinical, pathologic, and molecular risk stratification should be used to refine treatment regimens for children with ependymoma to reduce the risk of complications associated with therapy and increase the rate of disease control in the setting of combined modality or more intensive therapy. This review covers standards of care and current clinical trials for children with ependymoma, emphasizing the history and evolution of treatment regimens during the past 20 years and the clinical questions they hoped to address.
室管膜瘤是一种具有潜在转移能力的局部侵袭性肿瘤，可发生在儿童的大脑和脊髓的不同部位。肿瘤和治疗可能导致严重的发病率。由于生物学的多样性和对常规治疗的耐药性，许多患者仍然难以治愈。在过去的 20 年中，临床试验中系统地进行术后放疗，使许多患者通过客观的功能结果测量，达到了持久的疾病控制，从而提高了这一比例。临床、病理和分子风险分层应用于细化室管膜瘤患儿的治疗方案，以降低与治疗相关的并发症风险，并在联合治疗或更强化疗的情况下提高疾病控制率。这篇综述涵盖了儿童室管膜瘤的护理标准和当前的临床试验，强调了过去 20 年治疗方案的历史和演变，以及它们希望解决的临床问题。
