Clinical response to nivolumab in an INI1-deficient pediatric chordoma correlates with immunogenic recognition of brachyury.


Journal

NPJ precision oncology
ISSN: 2397-768X
Titre abrégé: NPJ Precis Oncol
Pays: England
ID NLM: 101708166

Informations de publication

Date de publication:
20 Dec 2021
Historique:
received: 06 04 2021
accepted: 22 10 2021
entrez: 21 12 2021
pubmed: 22 12 2021
medline: 22 12 2021
Statut: epublish

Résumé

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.

Identifiants

pubmed: 34931022
doi: 10.1038/s41698-021-00238-4
pii: 10.1038/s41698-021-00238-4
pmc: PMC8688516
doi:

Types de publication

Journal Article

Langues

eng

Pagination

103

Subventions

Organisme : Genome British Columbia
ID : 202SEQ
Organisme : Genome British Columbia
ID : 212SEQ
Organisme : Genome British Columbia
ID : B20POG
Organisme : Genome British Columbia
ID : 202SEQ
Organisme : Genome British Columbia
ID : 212SEQ
Organisme : Genome British Columbia
ID : B20POG
Organisme : Canada Foundation for Innovation (Fondation canadienne pour l'innovation)
ID : 33408
Organisme : Canada Foundation for Innovation (Fondation canadienne pour l'innovation)
ID : 20070
Organisme : Canada Foundation for Innovation (Fondation canadienne pour l'innovation)
ID : 30981
Organisme : Canada Foundation for Innovation (Fondation canadienne pour l'innovation)
ID : 30198
Organisme : Canada Foundation for Innovation (Fondation canadienne pour l'innovation)
ID : 36239
Organisme : Canada Foundation for Innovation (Fondation canadienne pour l'innovation)
ID : 36239
Organisme : Canada Foundation for Innovation (Fondation canadienne pour l'innovation)
ID : 20070
Organisme : Canada Foundation for Innovation (Fondation canadienne pour l'innovation)
ID : 30981
Organisme : Canada Foundation for Innovation (Fondation canadienne pour l'innovation)
ID : 33408
Organisme : Canada Foundation for Innovation (Fondation canadienne pour l'innovation)
ID : 35444
Organisme : Canada Foundation for Innovation (Fondation canadienne pour l'innovation)
ID : 30981
Organisme : Genome Canada (Génome Canada)
ID : 12002
Organisme : Genome Canada (Génome Canada)
ID : 12002

Informations de copyright

© 2021. Crown.

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Auteurs

Laura M Williamson (LM)

Canada's Michael Smith Genome Sciences Centre at BC Cancer, Vancouver, BC, Canada.

Craig M Rive (CM)

Canada's Michael Smith Genome Sciences Centre at BC Cancer, Vancouver, BC, Canada.

Daniela Di Francesco (D)

Canada's Michael Smith Genome Sciences Centre at BC Cancer, Vancouver, BC, Canada.

Emma Titmuss (E)

Canada's Michael Smith Genome Sciences Centre at BC Cancer, Vancouver, BC, Canada.

Hye-Jung E Chun (HE)

Canada's Michael Smith Genome Sciences Centre at BC Cancer, Vancouver, BC, Canada.

Scott D Brown (SD)

Canada's Michael Smith Genome Sciences Centre at BC Cancer, Vancouver, BC, Canada.

Katy Milne (K)

Deeley Research Centre, BC Cancer, Victoria, BC, Canada.
Department of Biochemistry and Microbiology, University of Victoria, Victoria, BC, Canada.

Erin Pleasance (E)

Canada's Michael Smith Genome Sciences Centre at BC Cancer, Vancouver, BC, Canada.

Anna F Lee (AF)

Department of Pathology and Laboratory Medicine, British Columbia Children's Hospital, Vancouver, BC, Canada.

Stephen Yip (S)

Department of Pathology and Laboratory Medicine, Vancouver General Hospital, Vancouver, BC, Canada.

Daniel G Rosenbaum (DG)

Department of Radiology, British Columbia Children's Hospital, Vancouver, BC, Canada.

Martin Hasselblatt (M)

Institute of Neuropathology, University Hospital Münster, Münster, Germany.

Pascal D Johann (PD)

Hopp Children's Cancer Center (KITZ), Heidelberg, Germany.
Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK) Core Center, Heidelberg, Germany.
Department of Pediatric Hematology and Oncology, University Hospital Heidelberg, Heidelberg, Germany.

Marcel Kool (M)

Hopp Children's Cancer Center (KITZ), Heidelberg, Germany.
Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK) Core Center, Heidelberg, Germany.
Princess Máxima Center for Pediatric Oncology, Utrecht, the Netherlands.

Melissa Harvey (M)

Division of Pediatric Hematology Oncology BMT, University of British Columbia, Vancouver, BC, Canada.

David Dix (D)

Division of Pediatric Hematology Oncology BMT, University of British Columbia, Vancouver, BC, Canada.

Daniel J Renouf (DJ)

Pancreas Centre BC, Vancouver, BC, Canada.
Department of Medical Oncology, BC Cancer, Vancouver, BC, Canada.

Robert A Holt (RA)

Canada's Michael Smith Genome Sciences Centre at BC Cancer, Vancouver, BC, Canada.
Department of Medical Genetics, University of British Columbia, Vancouver, BC, Canada.
Department of Molecular Biology and Biochemistry, Simon Fraser University, Burnaby, BC, Canada.

Brad H Nelson (BH)

Deeley Research Centre, BC Cancer, Victoria, BC, Canada.
Department of Biochemistry and Microbiology, University of Victoria, Victoria, BC, Canada.

Martin Hirst (M)

Canada's Michael Smith Genome Sciences Centre at BC Cancer, Vancouver, BC, Canada.
Department of Microbiology & Immunology, Michael Smith Laboratories, University of British Columbia, Vancouver, BC, Canada.

Steven J M Jones (SJM)

Canada's Michael Smith Genome Sciences Centre at BC Cancer, Vancouver, BC, Canada.
Department of Medical Genetics, University of British Columbia, Vancouver, BC, Canada.

Janessa Laskin (J)

Department of Medical Oncology, BC Cancer, Vancouver, BC, Canada.

Shahrad R Rassekh (SR)

Division of Pediatric Hematology Oncology BMT, University of British Columbia, Vancouver, BC, Canada.

Rebecca J Deyell (RJ)

Division of Pediatric Hematology Oncology BMT, University of British Columbia, Vancouver, BC, Canada.

Marco A Marra (MA)

Canada's Michael Smith Genome Sciences Centre at BC Cancer, Vancouver, BC, Canada. mmarra@bcgsc.ca.
Department of Medical Genetics, University of British Columbia, Vancouver, BC, Canada. mmarra@bcgsc.ca.

Classifications MeSH