ArticleClinical and translational radiation oncology2019
Radiation for ETMR: Literature review and case series of patients treated with proton therapy.
Article in Clinical and translational radiation oncology, 2019. 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, 47 citations in OpenAlex.
- Timing of radiotherapy and autologous stem cell transplantation in embryonal tumor with multilayered rosettes: a critical narrative review.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Review
- Recurrent C19MC-altered embryonal tumors with multilayered rosettes: outcomes of stereotactic radiosurgery.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2026Article
- Freiburg Neuropathology Case Conference: A 2-Year-Old Child with Progressive Headache, Nausea, and Personality Change.Clinical neuroradiology · 2026Article
- How is rosette formation in brain tumours linked with cerebrospinal fluid spread?Brain tumor pathology · 2026Review
- Outcomes following radiation therapy for embryonal tumor with multilayered rosettes (ETMR): results from the Pediatric Proton/Photon Consortium Registry (PPCR).Journal of neuro-oncology · 2025Article
- Review
- Evaluating the efficacy of radiotherapy in patients with embryonal tumor with multilayered rosettes: A systematic review and meta-analysis.Neuro-oncology practice · 2025Article
- Genetic mutation and immune infiltration in embryonal tumor with multilayered rosettes.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Article
- Integrated proteomics spotlight the proteasome as a therapeutic vulnerability in embryonal tumors with multilayered rosettes.Neuro-oncology · 2024Article
- Methylation-based Subclassifications of Embryonal Tumor with Multilayered Rosettes in Not Just Pediatric Brains.Experimental neurobiology · 2023Article
- Imaging of pediatric spine and spinal cord tumors: A COG Diagnostic Imaging Committee/SPR Oncology Committee/ASPNR White Paper.Pediatric blood & cancer · 2023Article
- Embryonal Tumors of the Central Nervous System with Multilayered Rosettes and Atypical Teratoid/Rhabdoid Tumors.Advances in experimental medicine and biology · 2023Article
- Clinical Management of Embryonal Tumor with Multilayered Rosettes: The CCMC Experience.Children (Basel, Switzerland) · 2022Article
- Embryonal and non-meningothelial mesenchymal tumors of the central nervous system - Advances in diagnosis and prognostication.Brain pathology (Zurich, Switzerland) · 2022Review
- Tandem autologous hematopoietic stem cell transplantation for embryonal brain tumors in infants and very young children.Bone marrow transplantation · 2022Article
- A surgical case of pediatric spinal medulloepithelioma.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2022Article
- Treatment of embryonal tumors with multilayered rosettes with carboplatin/etoposide induction and high-dose chemotherapy within the prospective P-HIT trial.Neuro-oncology · 2022Article
- Ependymoblastoma with pulmonary metastasis in an adolescent: A case report.Frontiers in neurology · 2022Article
- Utilizing preclinical models to develop targeted therapies for rare central nervous system cancers.Neuro-oncology · 2021Review
- Embryonal tumors with multi-layered rosettes: a disease of dysregulated miRNAs.Journal of neuro-oncology · 2020Review
Corrections and comments
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Authors and funding
8 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
background and purposeEmbryonal tumors with multilayered rosettes (ETMRs) are aggressive tumors that typically occur in young children. Radiation is often deferred or delayed for these patients due to late effects; proton therapy may mitigate some of these concerns. This study reviews the role of radiation in ETMR and describes initial results with proton therapy. MATERIALS AND
methodsRecords of patients with embryonal tumor with abundant neuropil and true rosettes (ETANTR), medulloepithelioma (MEP), and ependymoblastoma (EPL) treated with proton therapy at our institution were retrospectively reviewed. A literature review of cases of CNS ETANTR, MEP, and EPL published since 1990 was also conducted.
resultsSeven patients were treated with proton therapy. Their median age at diagnosis was 33 months (range 10-57 months) and their median age at radiation start was 42 months (range 17-58 months). Their median overall survival (OS) was 16 months (range 8-64 months), with three patients surviving 36 months or longer. Five patients had disease progression prior to starting radiation; all 5 of these patients failed in the tumor bed. A search of the literature identified 204 cases of ETMR with a median OS of 10 months (range 0.03-161 months). Median OS of 18 long-term survivors (≥36 months) in the literature was 77 months (range 37-184 months). Of these 18 long-term survivors, 17 (94%) received radiotherapy as part of their initial treatment; 14 of them were treated with craniospinal irradiation.
conclusionsOutcomes of patients with ETMR treated with proton therapy are encouraging compared to historical results. Further study of this rare tumor is warranted to better define the role of radiotherapy.
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