Gastrointestinal stromal tumors

Gastroenterol Clin North Am. 2013 Jun;42(2):399-415. doi: 10.1016/j.gtc.2013.01.001. Epub 2013 Mar 13.

Abstract

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumor of the gastrointestinal tract. Soon after GIST was recognized as a tumor driven by a KIT or platelet-derived growth factor receptor mutation, it became the first solid tumor target for tyrosine kinase inhibitor therapies. More recently, alternative molecular mechanisms for GIST pathogenesis have been discovered. These are related to deficiencies in the succinate dehydrogenase complex, NF1-gene alterations in connection with neurofibromatosis type 1 tumor syndrome, and mutational activation of the BRAF oncogene in very rare cases.

Publication types

  • Research Support, N.I.H., Intramural
  • Review

MeSH terms

  • Abdominal Pain / etiology
  • Gastrointestinal Hemorrhage / etiology*
  • Gastrointestinal Neoplasms / complications
  • Gastrointestinal Neoplasms / diagnosis*
  • Gastrointestinal Neoplasms / epidemiology
  • Gastrointestinal Neoplasms / genetics*
  • Gastrointestinal Neoplasms / therapy
  • Gastrointestinal Stromal Tumors / complications
  • Gastrointestinal Stromal Tumors / diagnosis*
  • Gastrointestinal Stromal Tumors / epidemiology
  • Gastrointestinal Stromal Tumors / genetics*
  • Gastrointestinal Stromal Tumors / therapy
  • Humans
  • Neurofibromatosis 1 / complications
  • Platelet-Derived Growth Factor / genetics*
  • Prognosis
  • Proto-Oncogene Proteins c-kit / genetics*
  • Succinate Dehydrogenase / deficiency

Substances

  • Platelet-Derived Growth Factor
  • platelet-derived growth factor A
  • Succinate Dehydrogenase
  • Proto-Oncogene Proteins c-kit