Ependimomas Intracraneales en Pediatría: enfoque clínico-quirúrgico

Abstract

Introduction: Ependymoma occupies the third place among brain tumors in Pediatrics, the infratentorial location being the most frequent. The best results were obtained with complete resections followed by radiation therapy. In young children, other therapeutic strategies are needed. The new WHO classification based on location, histopathology and molecular determinations will allow stratifying and adapting treatments.
The objective of this study is to identify clinical-surgical factors related to prognosis.
Materials and methods: Cross-sectional, observational and retrospective analysis, taking into account the age, sex, the percentage of resection and the evolution of patients with a diagnosis of intracranial ependymoma treated by the Neurosurgery and Oncology Departments of the HIAEP “Superiora Sor María Ludovica” between 2011 to 2020 and year 2020. The data were obtained from the analysis of medical records with prior consent.
Results: Twelve children with Ependymoma were included, 10 infratentorial. The mean age was 5.7 years and 83% were male. The supratentorials presented motor compromise and hemiparesis. They were completely resected in all the cases (2). The WHO III anaplastic subtype prevailed. The patient with the presence of RELA fusion received radiotherapy. They are both alive. At the infratentorial level, HTE predominated. Subtotal resection was achieved in 6 cases. The predominant subtype was WHO II. Those under 1 year of age received chemotherapy. All received local radiation therapy. 4 patients died, 1 due to metastatic recurrence, the rest due to post-surgical complications. Of the 6 living patients, the mean follow-up was 49.83 months, 2 received treatment for recurrence, being less than 1 year old at diagnosis and presenting the WHO III anaplastic subtype.
Conclusions: Younger age, infratentorial location, and anaplastic subtype are associated with higher morbidity and mortality. The degree of resection has a prognostic influence. Multidisciplinary intervention is required to design the best therapeutic strategy.