Format:
23
ISSN:
2589-0042
Content:
Malignant peripheral nerve sheath tumors (MPNSTs) are soft-tissue sarcomas of the peripheral nervous system that develop either sporadically or in the context of neurofibromatosis type 1 (NF1). MPNST diagnosis can be challenging and treatment outcomes are poor. We present here a resource consisting of the genomic characterization of 9 widely used human MPNST cell lines for their use in translational research. NF1-related cell lines recapitulated primary MPNST copy number profiles, exhibited NF1, CDKN2A, and SUZ12/EED tumor suppressor gene (TSG) inactivation, and presented no gain-of-function mutations. In contrast, sporadic cell lines collectively displayed different TSG inactivation patterns and presented kinase-activating mutations, fusion genes, altered mutational frequencies and COSMIC signatures, and different methylome-based classifications. Cell lines re-classified as melanomas and other sarcomas exhibited a different drug-treatment response. Deep genomic analysis, methylome-based classification, and cell-identity marker expression, challenged the identity of common MPNST cell lines, opening an opportunity to revise MPNST differential diagnosis.
Note:
Online verfügbar 31. Januar 2023, Artikelversion 9. Februar 2023
,
Gesehen am 10.07.2023
In:
iScience, Amsterdam : Elsevier, 2018, 26(2023), 2 vom: Feb., Artikel-ID 106096, Seite 1-23, 2589-0042
In:
volume:26
In:
year:2023
In:
number:2
In:
month:02
In:
elocationid:106096
In:
pages:1-23
In:
extent:23
Language:
English
DOI:
10.1016/j.isci.2023.106096
URL:
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