DLK1 Distinguishes Subsets of NF1-Associated Malignant Peripheral Nerve Sheath Tumors with Divergent Molecular Signatures.

ABSTRACT:

Purpose

Malignant peripheral nerve sheath tumor (MPNST) is the leading cause of premature death among individuals with neurofibromatosis type 1 (NF1), and the transcriptional aberrations that precede malignant transformation and contribute to MPNST tumorigenesis remain poorly defined. Alterations involving CDKN2A and components of PRC2 have been implicated as early drivers of peripheral nerve sheath tumor (PNST) evolution, but these events do not occur in all MPNST. Accordingly, emerging data have begun to highlight the importance of molecular-based stratification to improve outcomes in patients with NF1-PNST.

Experimental design

In this study, we perform an integrated analysis of multiple, independent datasets obtained from human patients with NF1 to gain critical insights

SUBMITTER: Mitchell DK 

PROVIDER: S-EPMC12081192 | biostudies-literature | 2025 May

REPOSITORIES: biostudies-literature