ArticleVirchows Archiv : an international journal of pathology2015
Long-term survival in a case of ETANTR with histological features of neuronal maturation after therapy.
Article in Virchows Archiv : an international journal of pathology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 29 citations in OpenAlex.
- Clinical, toxicity and long-term neurocognitive outcomes of first-line intensive chemotherapy in infant medulloblastoma: a single-center cohort study following AIEOP recommendations.Journal of neuro-oncology · 2025Article
- 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
- Cellular hierarchies of embryonal tumors with multilayered rosettes are shaped by oncogenic microRNAs and receptor-ligand interactions.Nature cancer · 2025Article
- Evaluating the efficacy of radiotherapy in patients with embryonal tumor with multilayered rosettes: A systematic review and meta-analysis.Neuro-oncology practice · 2025Article
- Integrated proteomics spotlight the proteasome as a therapeutic vulnerability in embryonal tumors with multilayered rosettes.Neuro-oncology · 2024Article
- Embryonal tumor with multilayered rosettes: Post-treatment maturation and implications for future therapy.Cancer reports (Hoboken, N.J.) · 2023Article
- Embryonal Tumors of the Central Nervous System with Multilayered Rosettes and Atypical Teratoid/Rhabdoid Tumors.Advances in experimental medicine and biology · 2023Article
- Molecular Landscape in Infant High-Grade Gliomas: A Single Center Experience.Diagnostics (Basel, Switzerland) · 2022Article
- Embryonal tumors with multilayered rosettes,Frontiers in oncology · 2022Article
- DNA methylation profiling as a model for discovery and precision diagnostics in neuro-oncology.Neuro-oncology · 2021Review
- Expanding the spectrum of EWSR1-PATZ1 rearranged CNS tumors: An infantile case with leptomeningeal dissemination.Brain pathology (Zurich, Switzerland) · 2021Article
- ETMR: a tumor entity in its infancy.Acta neuropathologica · 2020Review
- CT and Multimodal MR Imaging Features of Embryonal Tumors with Multilayered Rosettes in Children.AJNR. American journal of neuroradiology · 2019Article
- Radiation for ETMR: Literature review and case series of patients treated with proton therapy.Clinical and translational radiation oncology · 2019Article
- Embryonal tumor with multilayered rosettes: Overview of diagnosis and therapy.Neuro-oncology advancesArticle
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Authors and funding
9 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a rare subtype of primitive neuroectodermal tumors and one of the most aggressive brain tumors in children. The neoplasm harbors a specific genetic alteration, amplification of the miRNA cluster C19MC at 19q13.42. We report a case of a 21-month-old boy with a mass in the left fronto-opercular region. The lesion was partially resected and pathology examination revealed an ETANTR with immunoreativity for LIN28A protein and amplification of the C19MC locus. The child received the PNET infant indications followed by high-dose thiotepa which resulted in a significant reduction of the mass. Subsequently, a second operation was carried out and the residual mass removed. Histology at this time showed a low-grade lesion composed of neuronal cells ranging from neurocytes to ganglion cells embedded in abundant neuropil with no immature embryonal component and multilayered rosettes. In addition to these features, a decrease in the number of nuclei with amplification of the C19MC locus was also observed. Thirty-one months after the second operation, the patient is alive and well. Such long-term survival could be explained by neuronal maturation induced by therapy associated with reduction of neoplastic cells with amplification of C19MC locus. This case suggests that the induction of differentiation may represent an optimal treatment strategy for very aggressive malignancies as ETANTR.
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