ArticleNeuro-oncology2022
Treatment and outcome of intracranial ependymoma after first relapse in the 2nd AIEOP protocol.
Article in Neuro-oncology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 11 citations in OpenAlex.
- Patient-derived ependymoma and medulloblastoma tumoroids: generation, biobanking and drug screening.Nature protocols · 2026Review
- Clinical, surgical, and molecular features of pediatric supratentorial ependymomas: a single-institution experience.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Article
- Surgery and radiotherapy strategies at first relapse of pediatric intracranial ependymoma: an Italian retrospective study integrating molecular subgrouping.Journal of neuro-oncology · 2026Article
- Recurrent parietal lobe supratentorial ependymoma, ZFTA fusion-positive, CNS WHO grade 3, with new dural and calvarial reactive changes in a child: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Molecular Testing for the World Health Organization Classification of Central Nervous System Tumors: A Review.JAMA oncology · 2025Review
- Optimizing outcomes in intracranial ependymoma: a contemporary review.Frontiers in oncology · 2025Review
- Ependymoma from Benign to Highly Aggressive Diseases: A Review.Advances and technical standards in neurosurgery · 2024Review
- Patient- and xenograft-derived organoids recapitulate pediatric brain tumor features and patient treatments.EMBO molecular medicine · 2023Article
- 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
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Authors and funding
30 authors at 18 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundMore than 40% of patients with intracranial ependymoma need a salvage treatment within 5 years after diagnosis, and no standard treatment is available as yet. We report the outcome after first relapse of 64 patients treated within the 2nd AIEOP protocol.
methodsWe considered relapse sites and treatments, that is, various combinations of complete/incomplete surgery, if followed by standard or hypofractionated radiotherapy (RT) ± chemotherapy (CT). Molecular analyses were available for 38/64 samples obtained at first diagnosis. Of the 64 cases, 55 were suitable for subsequent analyses.
resultsThe median follow-up was 147 months after diagnosis, 84 months after first relapse, 5-year EFS/OS were 26.2%/30.8% (median EFS/OS 13/32 months) after relapse. For patients with a local relapse (LR), the 5-year cumulative incidence of second LRs was 51.6%, with a 5-year event-specific probability of being LR-free of 40.0%. Tumor site/grade, need for shunting, age above/below 3 years, molecular subgroup at diagnosis, had no influence on outcomes. Due to variation in the RT dose/fractionation used and the subgroup sizes, it was not possible to assess the impact of the different RT modalities. Multivariable analyses identified completion of surgery, the absence of symptoms at relapse, and female sex as prognostically favorable. Tumors with a 1q gain carried a higher cumulative incidence of dissemination after first relapse.
conclusionsSurvival after recurrence was significantly influenced by symptoms and completeness of surgery. Only a homogeneous protocol with well-posed, randomized questions could clarify the numerous issues, orient salvage treatment, and ameliorate prognosis for this group of patients.
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