ArticleNPJ precision oncology2021
Clinical response to nivolumab in an INI1-deficient pediatric chordoma correlates with immunogenic recognition of brachyury.
Article in NPJ precision oncology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
23 citing papers in PubMed, 32 citations in OpenAlex.
- Institutional experience and pooled survival analysis of chemotherapy based multimodal management in poorly differentiated chordoma.Discover oncology · 2026Article
- A patient-derived organoid model identifies TP53-dependent gemcitabine sensitivity in spinal chordoma.Discover oncology · 2026Article
- Immune and stromal signaling networks in chordoma: an evidence-weighted review of ligand-receptor interactions and therapeutic implications.Systematic reviews · 2026Review
- Integrated immune profiling of chordomas reveals spatially organized niches and functional heterogeneity.Neuro-oncology · 2026Article
- Comprehensive profiling of paediatric chordoma: poorly differentiated and conventional subtypes.Brain communications · 2026Article
- Complex immune microenvironment of chordoma: a road map for future treatment.Journal for immunotherapy of cancer · 2024Article
- Whole genome and transcriptome integrated analyses guide clinical care of pediatric poor prognosis cancers.Nature communications · 2024Article
- Chordoma Genetic Aberrations and Targeted Therapies Panorama: A Systematic Literature Review.Journal of clinical medicine · 2024Review
- Off-label prescribing of immune checkpoint inhibitor therapy at a single pediatric cancer center.Cancer medicine · 2024Article
- Multimodal profiling of chordoma immunity reveals distinct immune contextures.Journal for immunotherapy of cancer · 2024Article
- Dysregulation of noncoding RNA in chordoma; implications in identifying potential targets for novel therapeutic approaches.Molecular biology reports · 2024Review
- Role of immunotherapy in treatment refractory chordomas: review of current evidence.Frontiers in surgery · 2024Review
- Article
- Immune microenvironment and immunotherapy for chordoma.Frontiers in oncology · 2024Review
- Animal model considerations for chordoma research: reproducing the tumor microenvironmentFrontiers in oncology · 2024Review
- The NIH pediatric/young adult chordoma clinic and natural history study: Making advances in a very rare tumor.Pediatric blood & cancer · 2023Article
- Immunotherapy as a Promising Option for the Treatment of Advanced Chordoma: A Systemic Review.Cancers · 2022Review
- Metastatic skull base chordoma: A systematic review.Laryngoscope investigative otolaryngology · 2022Review
- A chronicle review of new techniques that facilitate the understanding and development of optimal individualized therapeutic strategies for chordoma.Frontiers in oncology · 2022Review
- Emerging target discovery and drug repurposing opportunities in chordoma.Frontiers in oncology · 2022Review
Corrections and comments
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Authors and funding
25 authors at 8 institutions in 3 countries.
Funding
Abstract
Poorly differentiated chordoma (PDC) is a recently recognized subtype of chordoma characterized by expression of the embryonic transcription factor, brachyury, and loss of INI1. PDC primarily affects children and is associated with a poor prognosis and limited treatment options. Here we describe the molecular and immune tumour microenvironment profiles of two paediatric PDCs produced using whole-genome, transcriptome and whole-genome bisulfite sequencing (WGBS) and multiplex immunohistochemistry. Our analyses revealed the presence of tumour-associated immune cells, including CD8+ T cells, and expression of the immune checkpoint protein, PD-L1, in both patient samples. Molecular profiling provided the rationale for immune checkpoint inhibitor (ICI) therapy, which resulted in a clinical and radiographic response. A dominant T cell receptor (TCR) clone specific for a brachyury peptide-MHC complex was identified from bulk RNA sequencing, suggesting that targeting of the brachyury tumour antigen by tumour-associated T cells may underlie this clinical response to ICI. Correlative analysis with rhabdoid tumours, another INI1-deficient paediatric malignancy, suggests that a subset of tumours may share common immune phenotypes, indicating the potential for a therapeutically targetable subgroup of challenging paediatric cancers.
Identifiers
What OpenQuestion holds
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.