ArticleNeuro-oncology2021
Second series by the Italian Association of Pediatric Hematology and Oncology of children and adolescents with intracranial ependymoma: an integrated molecular and clinical characterization with a long-term follow-up.
Article in Neuro-oncology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 38 citations in OpenAlex.
- SIOP Ependymoma I: Final results, long-term follow-up, and molecular analysis of the trial cohort-A BIOMECA Consortium Study.Neuro-oncology · 2022Trial
- A brief history of ependymoma.Neuro-oncology · 2026Review
- Developing an advanced risk stratification model for pediatric intracranial ependymoma based on the prospective trial E-HIT2000 and subsequent registries.Neuro-oncology · 2026Article
- Distinct relapse pattern across molecular ependymoma types.Neuro-oncology · 2025Article
- Optimizing outcomes in intracranial ependymoma: a contemporary review.Frontiers in oncology · 2025Review
- Ependymal Tumors: Overview of the Recent World Health Organization Histopathologic and Genetic Updates with an Imaging Characteristic.AJNR. American journal of neuroradiology · 2024Review
- Radiotherapy dosing in intracranial ependymoma using the national cancer database.Journal of neuro-oncology · 2024Article
- Secreted clusterin inhibits tumorigenesis by modulating tumor cells and macrophages in human meningioma.Neuro-oncology · 2024Article
- Pediatric Central Nervous System Tumor Overview and Emerging Treatment Considerations.Brain sciences · 2023Review
- A clinicopathological analysis of supratentorial ependymoma, ZFTA fusion-positive: utility of immunohistochemical detection of CDKN2A alterations and characteristics of the immune microenvironment.Brain tumor pathology · 2023Article
- Ependymomas.Pathologica · 2022Review
- 2021 WHO classification of tumours of the central nervous system: a review for the neuroradiologist.Neuroradiology · 2022Review
- Ependymoma: Evaluation and Management Updates.Current oncology reports · 2022Review
- Treatment and outcome of pediatric intracranial ependymoma after first relapse.Journal of neuro-oncology · 2022Article
- Essential Management of Pediatric Brain Tumors.Children (Basel, Switzerland) · 2022Review
- Treatment and outcome of intracranial ependymoma after first relapse in the 2nd AIEOP protocol.Neuro-oncology · 2022Article
- Outcomes after first relapse of children with intracranial ependymoma treated on the second l'Associazione Italiana di Ematologia e Oncologia Pediatrica (AIEOP) protocol.Neuro-oncology · 2022Article
- Toward Improved Diagnosis Accuracy and Treatment of Children, Adolescents, and Young Adults With Ependymoma: The International SIOP Ependymoma II Protocol.Frontiers in neurology · 2022Article
- Delineation of molecular characteristics in pediatric PFA ependymoma involving rare osseous and pulmonary metastases: A case report and literature review.Frontiers in oncology · 2022Article
- Polish Multi-Institutional Study of Children with Ependymoma-Clinical Practice Outcomes in the Light of Prospective Trials.Diagnostics (Basel, Switzerland) · 2021Article
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Authors and funding
30 authors at 14 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundA prospective 2002-2014 study stratified 160 patients by resection extent and histological grade, reporting results in 2016. We re-analyzed the series after a median of 119 months, adding retrospectively patients' molecular features.
methodsFollow-up of all patients was updated. DNA copy number analysis and gene-fusion detection could be completed for 94/160 patients, methylation classification for 68.
resultsProgression-free survival (PFS) and overall survival (OS) at 5/10 years were 66/58%, and 80/73%. Ten patients had late relapses (range 66-126 mo), surviving after relapse no longer than those relapsing earlier (0-5 y). On multivariable analysis a better PFS was associated with grade II tumor and complete surgery at diagnosis and/or at radiotherapy; female sex and complete resection showed a positive association with OS. Posterior fossa (PF) tumors scoring ≥0.80 on DNA methylation analysis were classified as PFA (n = 41) and PFB (n = 9). PFB patients had better PFS and OS. Eighteen/32 supratentorial tumors were classified as RELA, and 3 as other molecular entities (anaplastic PXA, LGG MYB, HGNET). RELA had no prognostic impact. Patients with 1q gain or cyclin-dependent kinase inhibitor 2A (CDKN2A) loss had worse outcomes, included significantly more patients >3 years old (P = 0.050) and cases of dissemination at relapse (P = 0.007).
conclusionsPreviously described prognostic factors were confirmed at 10-year follow-up. Late relapses occurred in 6.2% of patients. Specific molecular features may affect outcome: PFB patients had a very good prognosis; 1q gain and CDKN2A loss were associated with dissemination. To draw reliable conclusions, modern ependymoma trials need to combine diagnostics with molecular risk stratification and long-term follow-up.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.