Molecular classification of pediatric ependymomas

• Grant number: 16/23972-6
• Support type: Scholarships in Brazil - Master
• Effective date (Start): March 01, 2017
• Effective date (End): June 30, 2018
• Field of knowledge: Health Sciences - Medicine
• Cooperation agreement: Coordination of Improvement of Higher Education Personnel (CAPES)
• Principal Investigator: Luiz Gonzaga Tone
• Grantee: Graziella Ribeiro de Sousa
• Home Institution: Faculdade de Medicina de Ribeirão Preto (FMRP). Universidade de São Paulo (USP). Ribeirão Preto , SP, Brazil
• Associated research grant: 14/20341-0 - Interactions between emerging therapeutic targets and developmental pathways associated with tumorigenesis: emphasis on pediatric malignancies, AP.TEM

Abstract

Ependymoma (EPN) is a rare tumor of the central nervous system (CNS), which arises in ependymal cells. It is the third most frequent cerebral tumor in the childhood, corresponding approximately to 9% of the pediatric intracranial tumors. Currently, the treatment includes surgical resection and radiotherapy. Despite the therapeutic advances, 40% of the tumors are incurable and about 50% of the patients develop local recurrence. Some of the causes of therapeutic failure is the high tumor heterogeneity and the diagnostic contradiction with the clinical prognosis. In 2015, a study performed a molecular characterization of ependymomas, being described nine tumor subgroups with distinct profiles. These data emphasize the importance of a better stratification of EPNs, in order to apply specific therapies for each subgroup. Among the subgroups, the supratentorial RelA fusion EPN and the group A of posterior fossa EPN are more prevalent in children and are responsible for majority of mortality in this population. Studies focused on the classification of these two high risk subgroups are important for the understanding of the biology of this tumor and for the development of more effective therapies. The present study aims to establish a molecular classification of EPN samples from Pediatric Oncology service of the HC-FMRP and from Boldrini Children Center, in order to compare Brazilian patient's profile with clinical and epidemiological data from previous studies of EPN in global scale. (AU)