ArticleNeuro-oncology advances
A modified IRS-III chemotherapy regimen leads to prolonged survival in children with embryonal tumor with multilayer rosettes.
Article in Neuro-oncology advances. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.
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Who cites it
13 citing papers in PubMed, 23 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
- In vivo intratumoral heterogeneity in a dish: scalable forebrain organoid models of embryonal brain tumors for high-throughput personalized drug discovery.Cancer communications (London, England) · 2025Article
- Targeting B4GALT3 in BMSCs-EVs for Therapeutic Control of HCC via NF-κB pathway inhibition.Cell biology and toxicology · 2025Article
- Evaluating the efficacy of radiotherapy in patients with embryonal tumor with multilayered rosettes: A systematic review and meta-analysis.Neuro-oncology practice · 2025Article
- Molecular mechanism and therapeutic strategies for embryonal tumors with multilayered rosettes in children (Review).Molecular and clinical oncology · 2025Review
- Review
- Embryonal tumor with multilayered rosettes: Post-treatment maturation and implications for future therapy.Cancer reports (Hoboken, N.J.) · 2023Article
- Clinical Management of Embryonal Tumor with Multilayered Rosettes: The CCMC Experience.Children (Basel, Switzerland) · 2022Article
- Bromodomain and Extra-Terminal Protein Inhibitors: Biologic Insights and Therapeutic Potential in Pediatric Brain Tumors.Pharmaceuticals (Basel, Switzerland) · 2022Review
- 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
- ETMR: a tumor entity in its infancy.Acta neuropathologica · 2020Review
- LCLAT1 regulates cardiolipin composition, mitochondrial phenotype, Lin28A, and oncogenic signaling networks in ETMR.Neuro-oncology advancesArticle
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Authors and funding
8 authors at 7 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundEmbryonal tumor with multilayer rosettes (ETMR) is a rare CNS malignancy affecting young children that carries a very poor prognosis. Treatment with intensive surgical resection, radiotherapy, and high-dose chemotherapy is insufficient treatment in the vast majority of cases. Effective, biologically based therapies for this tumor are therefore desperately needed. The Dana-Farber Cancer Institute-modified IRS-III protocol incorporates preclinically active agents, such as doxorubicin and actinomycin D, into the treatment regimen for ETMR and may improve patient outcomes.
methodsThe authors present a case series of 5 children with ETMR treated with an IRS-III-based chemotherapy backbone.
resultsAll 5 patients received a gross-total tumor resection. Patients received between 12 and 51 weeks of IRS-III therapy at the discretion of their treating physician. Four patients received focal radiation therapy, with the fifth patient instead receiving a cycle of high-dose chemotherapy with autologous stem cell rescue. Four patients have progression-free survival of more than 18 months. Chemotherapy treatment was reasonably tolerated by all 5 patients with one case of mild sinusoidal obstructive syndrome and one case of Grade 3 peripheral neuropathy.
conclusionsThe patient outcomes in this small cohort are far better than would be expected based on the historical survival for this tumor. Given the tremendous need for effective therapy for ETMR, further investigation of this approach is warranted. An international consensus protocol based on the IRS-III regimen has been developed and will be available through a multicenter clinical trial and a global treatment registry.
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