Ependymoma in pediatric patients

Pediatric ependymomas encompass distinct tumor types characterized by variations in epigenetics, age distribution, localization, and prognosis. Histopathological features continue to hold relevance in risk stratification within these defined tumor types. The primary treatment approach involves achieving complete surgical removal, whenever feasible, utilizing intraoperative monitoring and neuronavigation. In cases where mandatory, a second surgery may be performed, ensued by adjuvant radiation therapy. Nevertheless, emerging proof suggests that certain ependymal tumors can be successfully treated with surgery only, whilst others may experience relapse despite adjuvant therapy. The function of chemotherapy remains unclear at present. Current therapeutic strategies yield affordable survival rates for most patients with ependymoma. The next hurdle is to surpass initial tumor control by employing risk-adapted therapy, aiming to minimize secondary effects and therapy-induced morbidity for low-risk individuals, while intensifying treatment for high-risk individuals. The recognition of specific variations may pave the way for targeted therapy, enabling personalized treatment approaches in the future.