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Aim: To evaluate the oncological outcomes and the prognostic factors for children with ependymoma who receive radiotherapy (RT) ± chemotherapy after surgery.
Material And Methods: The medical records of 71 children with ependymoma who received RT between 2001 and 2022 were retrospectively evaluated. Survival outcomes and prognostic factors were analyzed using log-rank and cox-regression tests. SPSS v24.0 was utilized for statistical analyses.
Results: Gross total resection (GTR) was achieved in 37 (52%) patients. Craniospinal fluid (CSF) seeding was observed in 8 (11%) patients at the time of diagnosis. The median RT dose was 54 Gy (42-60 Gy). The median time from surgery to the first RT was 2.4 months (1-109 months). The median follow-up time was 65.9 months (2.5-242.8 months), and 5-y overall survival, progression-free survival (PFS), and local recurrence-free survival (LRFS) were 74%, 39%, and 46%, respectively. Recurrence was observed in 41 (58%) patients. Among patients who initiated treatment with chemotherapy, 5-y PFS and LRFS were higher in patients who received RT at the time of diagnosis than those who received RT at the progression (23% vs. 0%, p < 0.001 and 39% vs 0%, p < 0.001). In multivariate analysis, increased time from surgery to radiotherapy was found to be a poor prognostic factor for PFS.
Conclusion: Young age, less than GTR, large residual tumor volume, initiation of treatment with chemotherapy after surgery, and increased time from surgery to radiotherapy may deteriorate survival. RT should not be delayed until progression, even in young patients receiving chemotherapy.
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http://dx.doi.org/10.5137/1019-5149.JTN.46386-24.2 | DOI Listing |
J Pathol Transl Med
September 2025
Department of Pathology, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Hwasun, Korea.
Central nervous system tumors with BCL6 corepressor (BCOR) internal tandem duplications (ITDs) constitute a rare, recently characterized pediatric neoplasm with distinct molecular and histopathological features. To date, 69 cases have been documented in the literature, including our institutional case. These neoplasms predominantly occur in young children, with the cerebellum representing the most frequent anatomical location.
View Article and Find Full Text PDFBrain Pathol
August 2025
Department of Pathology, Massachusetts General Hospital, Brigham and Women's Hospital, and Harvard Medical School, Boston, Massachusetts, USA.
The Consortium to Inform Molecular and Practical Approaches to Central Nervous System Tumor Taxonomy (cIMPACT-NOW) updates provide guidelines for the diagnosis of central nervous system (CNS) tumors and suggestions for future World Health Organization (WHO) classification. Following publication of the fifth edition WHO Classification of CNS Tumors (WHO CNS5) in 2021, the cIMPACT-NOW working group "Clarification" reviewed WHO CNS5 and prioritized two topics for further elucidation: (a) distinction of Glioblastoma, IDH-wildtype from Diffuse pediatric-type high-grade glioma, H3-wildtype, and IDH-wildtype and (b) clarification of subgroups of posterior fossa (PF) ependymal tumors. Recommendations regarding the IDH- and H3-wildtype diffuse high-grade gliomas include: (1) use caution assigning CNS WHO grade 4 (diagnosis of Glioblastoma, IDH-wildtype) to a "TERT promoter only", histologically low-grade, IDH-wildtype tumor; (2) EGFR gene amplification and +7/-10 chromosome copy number alterations should not be used as solitary defining features for diagnosing high-grade gliomas as Glioblastoma, IDH-wildtype in patients <40 years of age; (3) Diffuse pediatric-type high-grade glioma, H3-wildtype, and IDH-wildtype should be considered in the differential diagnosis in adults, especially those <40 years of age; (4) PDGFRA alteration, EGFR alteration, or MYCN amplification count as key molecular features of Diffuse pediatric-type high-grade glioma, H3-wildtype, and IDH-wildtype only in patients <25 years.
View Article and Find Full Text PDFChilds Nerv Syst
August 2025
Division of Neurosurgery, Department of Clinical Neurosciences, University of Cambridge, Cambridge, UK.
Objective: This study aims to understand the impact of the COVID-19 pandemic on the diagnosis, management, and clinical outcomes of children and young people (CYP) with brain tumours in England.
Design: UK-based retrospective observational study with two components. Nationwide brain tumour incidence data from the National Disease Registration Service was obtained (2015-2020) to study trends in newly diagnosed cases.
Nat Cell Biol
August 2025
Department of Developmental Neurobiology, St. Jude Children's Research Hospital, Memphis, TN, USA.
Over 95% of ependymomas that arise in the cortex are driven by a gene fusion involving the zinc finger translocation-associated (ZFTA) protein. Here, using super-resolution and lattice light-sheet microscopy, we demonstrate that the most frequent fusion variant, ZFTA-RELA (ZR), forms dynamic nuclear condensates that are required for oncogene expression and tumorigenesis. Mutagenesis studies of ZR reveal a key intrinsically disordered region (IDR) in RELA that governs condensate formation.
View Article and Find Full Text PDFMed Sci (Basel)
August 2025
Department of Neurology, Faculty of Medicine, Cairo University, Cairo 12613, Egypt.
Background: Ependymomas are primary CNS neoplasms that arise from the ependymal cells of the brain and spinal cord, accounting for 3-6% of all CNS tumors.
Aims: This study provides a comprehensive analysis of ependymoma survival patterns and examines non-cancer causes of death in the US.
Methods: This retrospective study used data from SEER 17 registries between 2000 and 2019 to evaluate the incidence of ependymoma, as well as the survival and mortality trends in the US.