A review of the biological and clinical implications of RAS-MAPK pathway alterations in neuroblastoma

J Exp Clin Cancer Res. 2021 Jun 8;40(1):189. doi: 10.1186/s13046-021-01967-x.


Neuroblastoma is the most common extra-cranial solid tumor in children, representing approximately 8% of all malignant childhood tumors and 15% of pediatric cancer-related deaths. Recent sequencing and transcriptomics studies have demonstrated the RAS-MAPK pathway's contribution to the development and progression of neuroblastoma. This review compiles up-to-date evidence of this pathway's involvement in neuroblastoma. We discuss the RAS-MAPK pathway's general functioning, the clinical implications of its deregulation in neuroblastoma, and current promising therapeutics targeting proteins involved in signaling.

Keywords: ALK; ERK1/2; Inhibitors; MEK1/2; Neuroblastoma; RAS; RAS-MAPK; RASopathie.

Publication types

  • Review

MeSH terms

  • Humans
  • MAP Kinase Signaling System / genetics*
  • Neuroblastoma / genetics*