The molecular landscape of well differentiated retroperitoneal liposarcoma.


Journal

The Journal of pathology
ISSN: 1096-9896
Titre abrégé: J Pathol
Pays: England
ID NLM: 0204634

Informations de publication

Date de publication:
10 2021
Historique:
revised: 14 06 2021
received: 22 05 2021
accepted: 18 06 2021
pubmed: 23 6 2021
medline: 1 1 2022
entrez: 22 6 2021
Statut: ppublish

Résumé

Well differentiated liposarcoma (WD-LPS) is a relatively rare tumour, with fewer than 50 cases occurring per year in the UK. These tumours are both chemotherapy- and radiotherapy-resistant and present a significant treatment challenge requiring radical surgery. Little is known of the molecular landscape of these tumours and no current targets for molecular therapy exist. We aimed to carry out a comprehensive molecular characterisation of WD-LPS via whole genome sequencing, RNA sequencing, and methylation array analysis. A recurrent mutation within exon 1 of FOXD4L3 was observed (chr9:70,918,189A>T; c.322A>T; p.Lys108Ter). Recurrent mutations were also observed in Wnt signalling, immunity, DNA repair, and hypoxia-associated genes. Recurrent amplification of HGMA2 was observed, although this was in fact part of a general amplification of the region around this gene. Recurrent gene fusions in HGMA2, SDHA, TSPAN31, and MDM2 were also observed as well as consistent rearrangements between chromosome 6 and chromosome 12. Our study has demonstrated a recurrent mutation within FOXD4L3, which shows evidence of interaction with the PAX pathway to promote tumourigenesis. © 2021 The Authors. The Journal of Pathology published by John Wiley & Sons, Ltd. on behalf of The Pathological Society of Great Britain and Ireland.

Identifiants

pubmed: 34156092
doi: 10.1002/path.5749
doi:

Substances chimiques

FOXD4 protein, human 0
Forkhead Transcription Factors 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

132-140

Subventions

Organisme : Medical Research Council
ID : MR/M016587/1
Pays : United Kingdom
Organisme : Cancer Research UK
ID : C31641/A23923
Pays : United Kingdom
Organisme : Wellcome Trust
ID : 102732/Z/13/Z
Pays : United Kingdom

Informations de copyright

© 2021 The Authors. The Journal of Pathology published by John Wiley & Sons, Ltd. on behalf of The Pathological Society of Great Britain and Ireland.

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Auteurs

Robert Tyler (R)

Institute of Cancer and Genomic Medicine, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.

Mark P Dilworth (MP)

Institute of Cancer and Genomic Medicine, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.

Jonathan James (J)

Institute of Cancer and Genomic Medicine, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.

Daniel Blakeway (D)

Institute of Cancer and Genomic Medicine, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.

Joanne D Stockton (JD)

Institute of Cancer and Genomic Medicine, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.

Dion G Morton (DG)

Institute of Cancer and Genomic Medicine, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.

Phillipe Taniere (P)

Midland Abdominal Retroperitoneal Sarcoma Unit (MARSU), University Hospital Birmingham, Birmingham, UK.

David Gourevitch (D)

Midland Abdominal Retroperitoneal Sarcoma Unit (MARSU), University Hospital Birmingham, Birmingham, UK.

Anant Desai (A)

Midland Abdominal Retroperitoneal Sarcoma Unit (MARSU), University Hospital Birmingham, Birmingham, UK.

Andrew D Beggs (AD)

Institute of Cancer and Genomic Medicine, College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK.

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