Quadruple wild-type (WT) GIST: defining the subset of GIST that lacks abnormalities of KIT, PDGFRA, SDH, or RAS signaling pathways

Cancer Med. 2015 Jan;4(1):101-3. doi: 10.1002/cam4.325. Epub 2014 Aug 28.

Abstract

A subset of GISTs lack mutations in the KIT/PDGFRA or RAS pathways and yet retain an intact succinate dehydrogensase (SDH) complex. We propose that these KIT/PDGFRA/SDH/RAS-P WT GIST tumors be designated as quadruple wild-type (WT) GIST. Further molecular and clinicophatological characterization of quadruple WT GIST will help to determine their prognosis as well as assist in the optimization of medical management, including clinical test of novel therapies.

Keywords: BRAF; GIST; NF-1; RAS; SDH deficiency; SDHA; SDHB; gastrointestinal stromal tumors; quadruple WT; quadruple negative; wild type.

Publication types

  • Letter
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Gastrointestinal Stromal Tumors / diagnosis*
  • Gastrointestinal Stromal Tumors / genetics*
  • Gastrointestinal Stromal Tumors / metabolism
  • Humans
  • Mutation
  • Proto-Oncogene Proteins c-kit / genetics*
  • Proto-Oncogene Proteins c-kit / metabolism
  • Proto-Oncogene Proteins p21(ras) / genetics*
  • Proto-Oncogene Proteins p21(ras) / metabolism
  • Receptor, Platelet-Derived Growth Factor alpha / genetics*
  • Receptor, Platelet-Derived Growth Factor alpha / metabolism
  • Signal Transduction
  • Succinate Dehydrogenase / genetics*
  • Succinate Dehydrogenase / metabolism

Substances

  • Succinate Dehydrogenase
  • Proto-Oncogene Proteins c-kit
  • Receptor, Platelet-Derived Growth Factor alpha
  • Proto-Oncogene Proteins p21(ras)