Molecular pathobiology of gastrointestinal stromal sarcomas

Research output: Chapter in Book/Report/Conference proceedingChapter

288 Scopus citations


Gastrointestinal stromal tumors (GISTs) form an interesting group of sarcomas whose unique pathobiology provides a model of how molecularly targeted therapeutics can have a major impact on patient welfare. Approximately 85% of GISTs are driven by oncogenic mutations in either of two receptor tyrosine kinases: KIT or platelet-derived growth factor receptor α. We review the pivotal relationship between specific mutations in these kinase genes, the origin and pathologic spectrum of GISTs, and the response of these tumors to treatment with kinase inhibitors such as imatinib and sunitinib. Mechanisms of resistance to kinase inhibitor therapy are discussed, and targets for the next generation of therapeutics are considered. The rapid evolution in our understanding of GISTs, which stems directly from the close alliance of basic and clinical researchers in the field, illustrates the growing role of the molecular classification of solid tumors in the development of modern oncological treatments.

Original languageEnglish (US)
Title of host publicationAnnual Review of Pathology
Subtitle of host publicationMechanisms of Disease
Number of pages30
StatePublished - 2008

Publication series

NameAnnual Review of Pathology: Mechanisms of Disease
ISSN (Print)1553-4006
ISSN (Electronic)1553-4014


  • Gist
  • Imatinib
  • Sunitinib
  • Tyrosine kinase inhibitor

ASJC Scopus subject areas

  • Pathology and Forensic Medicine


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