From the Guidelines
Surgery is the crucial initial treatment for ependymomas, followed by local radiotherapy in pediatric patients with intracranial ependymomas of WHO grades II or III, regardless of residual tumor volume, as recommended by the eano guidelines for the diagnosis and treatment of ependymal tumors 1. The diagnosis and disease staging of ependymomas are performed by craniospinal MRI, and tumor classification is achieved by histological and molecular diagnostic assessment of tissue specimens according to the World Health Organization (WHO) classification 2016 1. Key considerations in the management of ependymomas include:
- The level of evidence for diagnostic and therapeutic interventions is higher in the pediatric compared with the adult patient population 1
- Tumor classification into WHO grades I, II, and III, with distinct genetic and epigenetic alterations, and distinct outcomes 1
- The importance of gross total resection as the mainstay of treatment in spinal ependymomas, with radiotherapy reserved for incompletely resected tumors 1
- The use of chemotherapy alone in young children <12 months and for adults with recurrent disease when further surgery and irradiation are no longer feasible 1 The prognosis for ependymoma patients depends on several factors, including the tumor's location, extent of surgical removal, grade, and the patient's age, with complete surgical resection being the most important prognostic factor 1.
From the Research
Overview of Ependymoma
- Ependymomas are rare primary central nervous system tumors occurring in children and young adults, which can be indolent or locally aggressive depending on location, histology, and extent of resection 2.
- They comprise about 10% of all pediatric brain tumors, with the most consistent prognostic factor for cure being the extent of surgical resection 3.
Treatment Options
- Treatment involves maximal surgical resection and usually focal radiation therapy, depending on the presence of residual disease and tumor grade 2, 3.
- Chemotherapy has been studied for both adults and children but does not have an established role in adjuvant therapy, although it can be considered in certain cases, such as residual disease after surgery or for salvage therapy 2, 3, 4.
- Conformal radiotherapy is recommended for grade 3 or incompletely resected grade II tumors, with proton therapy increasingly employed, especially in children, to reduce the risk of neurocognitive and endocrine sequelae 5, 6.
Prognostic Factors
- The extent of surgical resection is a major prognostic factor, with complete resection associated with better outcomes 3, 6.
- Tumor grade, location, and presence of certain molecular alterations, such as RELA fusion or chromosome 1q gain, can also impact prognosis 5, 6.
- Age is not a significant prognostic factor, with similar event-free survival rates for patients with ependymoma younger than 3 years of age and older patients who receive immediate postoperative CRT 5.
Current Research and Future Directions
- Ongoing studies are investigating the use of novel molecularly targeted therapies to improve outcomes and reduce toxicity for patients with ependymoma 4.
- Refinements in patient stratification schemes based on clinical variables and molecular profiles are also being explored to improve treatment outcomes 4.
- The discovery of new druggable pathways may increase the therapeutic armamentarium for ependymoma in the near future 6.