EPEN-02. EVALUATION OF TREATMENT OUTCOMES AND EXPRESSION OF EMT-RELATED TRANSCRIPTION FACTORS AS NOVEL THERAPEUTIC TARGETS IN PEDIATRIC EPENDYMOMA

Abstract

OBJECTIVEIntracranial ependymomas are common brain tumors in children. However, prognosis, especially in young children, remains poor because of the chemo- and radioresistant properties of intracranial ependymomas. Furthermore, effective treatments for intracranial ependymomas remain a challenge. The epithelial-to-mesenchymal transition (EMT) is important for invasion and metastasis in many cancers. This study aimed to evaluate and compare treatment outcomes with the expression of EMT-related transcription factors in pediatric ependymomas.MATERIAL AND METHODSMedical and radio-imaging data of 22 (11 boys, 11 girls) patients aged <15 years with intracranial ependymomas were reviewed from January 1983 to December 2018. Six cases were subdivided into clinicopathological-molecular subgroups and immunohistochemically analyzed for Slug and ZEB.RESULTSThe median age at the start of treatment was 5 years (range 8 months–15 years) (9 cases were aged <3 years). The median progression-free survival (PFS) was 25.6 (range, 0.8–383.5) months; the median overall survival (OS) was 81.9 (range, 2.9–383.5) months. Extent of resection and malignant histology were significant prognostic factors for OS and PFS in multivariate analysis. There were 6 cases (2 cases of PFA, 2 of PFB, 1 of ST and 1 case of ST-RELA). Nuclear expression of ZEB1 was found in all tumors; however, that of Slug increased only in PFA and PFB tumors, which were associated with a poor prognosis.CONCLUSIONExpression of EMT-related transcription factors was increased in pediatric ependymomas. These data suggest that EMT is a novel therapeutic target for treating pediatric intracranial ependymomas.