ArticleOncotarget2017
Personalized therapy: CNS HGNET-BCOR responsiveness to arsenic trioxide combined with radiotherapy.
Article in Oncotarget, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
16 citing papers in PubMed, 1 synthesis or guideline pooled it, 30 citations in OpenAlex.
- CNS tumor with EP300::BCOR fusion: discussing its prevalence in adult population.Acta neuropathologica communications · 2023Pooled it
- The future of targeted therapies for pediatric central nervous system tumors.Neuro-oncology advances · 2026Review
- BCOR loss promotes both retinoblastoma growth and susceptibility to IGF1R inhibition.Neuro-oncology · 2025Article
- Pediatric high-grade neuroepithelial BCOR tandem duplicated tumor: An illustrative case.Surgical neurology international · 2025Article
- A novel KDM2A::YAP1 fusion in a pediatric supratentorial CNS neoplasm resembling a tumor with BCOR internal tandem duplication.Free neuropathology · 2025Article
- The cGAS/STING/IFN-1 Response in Squamous Head and Neck Cancer Cells after Genotoxic Challenges and Abrogation of the ATR-Chk1 and Fanconi Anemia Axis.International journal of molecular sciences · 2023Article
- Article
- Imaging Features with Histopathologic Correlation of CNS High-Grade Neuroepithelial Tumors with aAJNR. American journal of neuroradiology · 2022Article
- Review
- BCOR Internal Tandem Duplication Expression in Neural Stem Cells Promotes Growth, Invasion, and Expression of PRC2 Targets.International journal of molecular sciences · 2021Article
- Central nervous system high-grade neuroepithelial tumor with BCOR alteration (CNS HGNET-BCOR)-case-based reviews.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2020Review
- Arsenic Trioxide exerts cytotoxic and radiosensitizing effects in pediatric Medulloblastoma cell lines of SHH Subgroup.Scientific reports · 2020Article
- Ceritinib-Induced Regression of an Insulin-Like Growth Factor-Driven Neuroepithelial Brain Tumor.International journal of molecular sciences · 2019Article
- IGF1R Is a Potential New Therapeutic Target for HGNET-BCOR Brain Tumor Patients.International journal of molecular sciences · 2019Article
- Inhibition of the hedgehog pathway for the treatment of cancer using Itraconazole.OncoTargets and therapy · 2019Article
- Role of GLI Transcription Factors in Pathogenesis and Their Potential as New Therapeutic Targets.International journal of molecular sciences · 2018Review
Corrections and comments
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Authors and funding
29 authors at 6 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
High-grade neuroepithelial tumor of the central nervous system with BCOR alteration (HGNET-BCOR) is a rare, highly malignant tumor. At the time of this publication, no standard protocol exists to treat this tumor entity. In this work, we tested the responsiveness of the primary culture PhKh1 derived from tumor tissue from a pediatric HGNET-BCOR patient (P1) to inhibitors of the Sonic hedgehog pathway combined with radiation. The SMO inhibitors vismodegib and itraconazole had low effect on the proliferation of the PhKh1 cells. However, the GLI inhibitor arsenic trioxide reduced the expression of GLI target genes in the PhKh1 cells and in combination with radiotherapy significantly decreased their clonogenic potential. PhKh1 cells resistant to arsenic trioxide were characterized by the overexpression of molecular chaperones. We combined arsenic trioxide and radiation in the relapse therapy protocol of P1, achieving complete remission after seven weeks. Clinical remission lasted for six months, when P1 developed systemic metastases. Meanwhile, an increase in the concentration of circulating tumor DNA carrying a BCOR internal tandem duplication was observed. Molecular characterization of a second patient (P2) was also performed. In P2, we detected a larger tandem duplication and greater activation of the Sonic hedgehog pathway than in P1. These findings suggest that combining arsenic trioxide with radiotherapy may represent a new therapeutic approach. Moreover, peripheral blood analysis for circulating tumor DNA could help in the early detection of systemic metastases.
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Registered trials
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.