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.

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