Establishment, characterization and functional testing of two novel ex vivo extraskeletal myxoid chondrosarcoma (EMC) cell models

Extraskeletal myxoid chondrosarcoma (EMC) is a malignant mesenchymal neoplasm of uncertain differentiation as classified by the WHO Classification of Tumours 2020. Although often associated with pronlonged survival, EMC has high rates of distant recurrences and disease-associated death. EMCs are tra...

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Published in:Human cell : official journal of Human Cell Research Society 2023-01, Vol.36 (1), p.446-455
Main Authors: Bangerter, Jana Lucia, Harnisch, Kim Jannis, Chen, Yanjiang, Hagedorn, Catherine, Planas-Paz, Lara, Pauli, Chantal
Format: Article
Language:English
Subjects:
Antitumor agents
Biomedical and Life Sciences
Cell Biology
Cell culture
Cell Line
Chondrosarcoma
Chondrosarcoma - genetics
Chondrosarcoma - metabolism
Chondrosarcoma - pathology
Disease
DNA fingerprinting
DNA methylation
DNA sequencing
Extraskeletal myxoid chondrosarcoma
Gene rearrangement
Gynecology
Humans
Life Sciences
Medical prognosis
Mesenchyme
Neoplasia
Neoplasms, Connective and Soft Tissue - genetics
Oncology
Reproductive Medicine
Sarcoma
Soft Tissue Neoplasms - genetics
Stem Cells
Surgery
Tumors
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Summary:Extraskeletal myxoid chondrosarcoma (EMC) is a malignant mesenchymal neoplasm of uncertain differentiation as classified by the WHO Classification of Tumours 2020. Although often associated with pronlonged survival, EMC has high rates of distant recurrences and disease-associated death. EMCs are translocation sarcomas and harbor in > 90% of the cases an NR4A3 rearrangement. The molecular consequences of the NR4A3 gene fusions are not yet fully elucidated as well-characterized ex vivo cell models for EMC are lacking. Patient-derived ex vivo models are important and essential tools for investigating disease mechanisms associated with diseases that are rare, that exhibit poor prognosis and for the identification of potential novel treatment options. We established two novel EMC ex vivo models ( USZ20-EMC1 and USZ22-EMC2 ) for functional testing and research purposes. USZ20-EMC1 and USZ22-EMC2 were established and maintained as sarco-sphere cell models for several months in culture. The cells were molecularly characterized using DNA sequencing and methylation profiling. Both cell models represent their native tumor tissue as confirmed by histomorphology and their molecular profiles, suggesting that native tumor cell function can be recapitulated in the ex vivo models. Using a functional screening approach, novel anti-cancer drug sensitivities including potential synergistic combinations were identified. In conclusion, two novel EMC ex vivo cell models ( USZ20-EMC1 and USZ22-EMC2 ) were successfully established and characterized from native tumor tissues. Both cell models will be useful tools for further investigating disease mechanisms and for answering basic and translational research questions.
ISSN:1749-0774
0914-7470
1749-0774
DOI:10.1007/s13577-022-00818-x