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. Author manuscript; available in PMC: 2020 Jul 1.
Published in final edited form as: Mol Cancer Res. 2019 Apr 25;17(7):1417–1428. doi: 10.1158/1541-7786.MCR-19-0147

Figure 1. MPNST Pathogenesis.

Figure 1.

Neoplastic growth in the nerves of an NF-1 patient is initiated after loss of the normal NF1 allele in an SPC. The ensuing paracrine signaling recruits fibroblasts, macrophages, and mast cells to the growing tumor. These tumors are termed plexiform neurofibromas (PNs), due to their heterogeneous composition. They develop in 50% of NF-1 patients. Typically, PNs only grow through adolescence. Further tumor growth in adulthood is observed to the context of ANF and MPNSTs. Upon loss of additional tumor suppressors (p53, CDKN2A, SUZ12, EED) in an NF1 null SC or SPC, SCs can transform into MPNST. This occurs in about 10% of NF-1 patients. These tumors metastasize readily and recur frequently after removal. High grade tumors are characterized by loss of H3K27me3. This figure was created in part from Servier Medical Art.