Response criteria for paediatric intracranial ependymoma vary historically and across different international cooperative groups. The Response Assessment in the Pediatric Neuro-Oncology (RAPNO) working group, consisting of an international panel of paediatric and adult neuro-oncologists, neuro-radiologists, radiation oncologists, and neurosurgeons, was established to address both the issues and the unique challenges in assessing the response in children with CNS tumours. We established a subcommittee to develop response assessment criteria for paediatric ependymoma. Current practice and literature were reviewed to identify major challenges in assessing the response of paediatric ependymoma to clinical trial therapy. For areas in which data were scarce or unavailable, consensus was reached through an iterative process. RAPNO response assessment recommendations include assessing disease response on the basis of changes in tumour volume, and using event-free survival as a study endpoint for patients entering clinical trials without bulky disease. Our recommendations for response assessment include the use of brain and spine MRI, cerebral spinal fluid cytology, neurological examination, and steroid use. Baseline postoperative imaging to assess for residual tumour should be obtained 24–48 h after surgery. Our consensus recommendations and response definitions should be prospectively validated in clinical trials.

儿科颅内室管膜瘤的反应标准在历史上和不同的国际合作组织之间有所不同。小儿神经肿瘤学 (RAPNO) 工作组的反应评估由一个由儿科和成人神经肿瘤学家、神经放射学家、放射肿瘤学家和神经外科医生组成的国际小组组成，旨在解决这些问题和独特的挑战。评估患有 CNS 肿瘤的儿童的反应。我们成立了一个小组委员会来制定小儿室管膜瘤的反应评估标准。回顾了当前的实践和文献，以确定评估小儿室管膜瘤对临床试验治疗的反应的主要挑战。对于数据稀缺或不可用的领域，通过迭代过程达成共识。RAPNO 反应评估建议包括根据肿瘤体积的变化评估疾病反应，并使用无事件生存期作为进入临床试验且没有大块疾病的患者的研究终点。我们对反应评估的建议包括使用脑和脊柱 MRI、脑脊液细胞学检查、神经系统检查和类固醇使用。术后 24-48 小时应获得用于评估残留肿瘤的基线术后影像。我们的共识建议和反应定义应在临床试验中进行前瞻性验证。我们对反应评估的建议包括使用脑和脊柱 MRI、脑脊液细胞学检查、神经系统检查和类固醇使用。术后 24-48 小时应获得用于评估残留肿瘤的基线术后影像。我们的共识建议和反应定义应在临床试验中进行前瞻性验证。我们对反应评估的建议包括使用脑和脊柱 MRI、脑脊液细胞学检查、神经系统检查和类固醇使用。术后 24-48 小时应获得用于评估残留肿瘤的基线术后影像。我们的共识建议和反应定义应在临床试验中进行前瞻性验证。