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. 2020 Jun 24;11(6):691. doi: 10.3390/genes11060691

Figure 1.

Figure 1

Signaling pathways altered due to genetic changes observed in malignant peripheral nerve sheath tumors (MPNST). The most common alterations in MPNST are loss of function of multiple tumor suppressors including NF1, p16/CDKN2A, TP53, and SUZ12/EED. Loss of NF1, as well as epigenetic changes due to loss of PRC2 components, leads to increased signaling through the RAS/RAF/MEK and PI3K/AKT pathways. Additional molecular events observed in subsets of MPNST include mutations in BRAF, amplification of EGFR or MET receptor tyrosine kinases (RTKs), and changes to chromatin structure through mutations in alpha thalassemia/mental retardation syndrome X (ATRX) and other epigenetic modifiers. EGF/EGFR = epidermal growth factor/receptor; PDGF/PDGFR = platelet derived growth factor/receptor; HGF = hepatocyte growth factor; ERK = extracellular signal regulated kinase; CDK = cyclin dependent kinase; RB = retinoblastoma; TF = transcription factor; ER = endoplasmic reticulum.