Establishment and characterization of a patient-derived metastatic extraskeletal Ewing sarcoma cell line ES-ZSS-1.
Sarcoma, Ewing
/ genetics
Humans
Cell Line, Tumor
Epithelial-Mesenchymal Transition
/ genetics
Mutation
Twist-Related Protein 1
/ genetics
RNA-Binding Protein EWS
/ genetics
Nuclear Proteins
/ genetics
Oncogene Proteins, Fusion
/ genetics
Animals
Neoplasm Metastasis
/ genetics
Proto-Oncogene Protein c-fli-1
/ genetics
Bone Neoplasms
/ genetics
Heterografts
Cohesins
Epithelial-to-mesenchymal transition
Extraskeletal Ewing sarcoma
Genomic characteristic
Metastatic and drug resistance
Patient-derived cancer cell line
Journal
Human cell
ISSN: 1749-0774
Titre abrégé: Hum Cell
Pays: Japan
ID NLM: 8912329
Informations de publication
Date de publication:
30 Oct 2024
30 Oct 2024
Historique:
received:
10
07
2024
accepted:
04
10
2024
medline:
31
10
2024
pubmed:
30
10
2024
entrez:
30
10
2024
Statut:
epublish
Résumé
The methods available for treating metastatic Ewing sarcoma (ES) are inadequate; thus, innovative therapeutic approaches need to be developed. However, the lack of clinically relevant ES models has hindered the discovery of drugs for this disease. In this study, we established and characterized a patient-derived xenograft (PDX) cell line model, which was constructed using tumor tissue from a patient with metastatic extraskeletal ES. The cells were found to recapitulate the morphological and histopathological features of the patient tumor and were designated as ES-ZSS-1. The cells harbor the characteristic EWSR1-FLI1 infusion and underwent successive passages in vitro. By performing gene expression profiling, we found that the mutation in STAG2 was the most frequent. An increase in Twist1 and epithelial-to-mesenchymal transition (EMT) was recorded. These genetic features might be relevant to metastasis and resistance to chemotherapy. To summarize, the novel patient-derived ES cell line we developed closely mimics the phenotype and genotype of patient tumors, making it a reliable tool for research on metastatic ES.
Identifiants
pubmed: 39475964
doi: 10.1007/s13577-024-01133-3
pii: 10.1007/s13577-024-01133-3
doi:
Substances chimiques
STAG2 protein, human
0
Twist-Related Protein 1
0
TWIST1 protein, human
0
RNA-Binding Protein EWS
0
Nuclear Proteins
0
Oncogene Proteins, Fusion
0
Proto-Oncogene Protein c-fli-1
0
EWSR1-FLI1 fusion protein, human
0
Cohesins
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
12Informations de copyright
© 2024. The Author(s) under exclusive licence to Japan Human Cell Society.
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