부산대학교 도서관

The classification of intracranial ependymomas became segmented with the introduction of WHO2021 classification. The annual incidence of pediatric (age 15 years or younger) intracranial ependymomas in Japan is estimated to be about 60 cases. It is not easy to establish the best treatment strategy for this rare disease on the basis of WHO2021 classification. Only one peer-reviewed paper on the guideline for this disease was published by the European Association of Neuro-Oncology (EANO) in 2018. This guideline emphasized the importance of radiation therapy immediately after surgical resection in children older than 12 months. In Japan, the guideline was published on the web page of the Japan Society for Neuro-Oncology in September 2021. In this guideline, radiation therapy was not strongly recommended for patients under 3 years of age. At this point, two multicenter randomized clinical trials, which are primarily intended to determine the efficacy of chemotherapy for this disease, are in progress. In years to come, the following points should be resolved: (1) further improvement of treatment results after total resection of the tumor, (2) verification of efficacy of chemotherapy, (3) determination of the most suitable definitive age of initiation into radiation, and (4) selection of an appropriate treatment strategy depending on molecular classification.