Pathology of the Gastrointestinal Tract

2017 Edition
| Editors: Fátima Carneiro, Paula Chaves, Arzu Ensari

Gastrointestinal Stromal Tumor

Reference work entry
DOI: https://doi.org/10.1007/978-3-319-40560-5_1619

Synonyms

GIST is the English acronym for Gastro Intestinal Stromal Tumor

Definition

It is a mesenchymal tumor, generally with immunohistochemistry expression of CD117-cluster of differentiation molecule 117/KIT (kinase-tyrosine receptor of stem cell factor), driven frequently by KIT or PDGFA (platelet-derived growth factor alpha) activation mutations, that occurs along the gastrointestinal tract, rarely extra-gastrointestinal tract (E-GIST), and displays a spectrum of benign to malignant clinical behavior.

Clinical Features

  • Incidence

    Annual incidence of GISTs is 11–19.6/million population (Corless et al. 2011).

  • Age

    GISTs occur typically in middle age to old adults (median age: ∼60–65 years), and rarely in childhood and young adults (Miettinnen et al. 2010).

  • Sex

    No distinct differences in incidence by gender, probably slightly more common in males.

  • Site

    GISTs occur most commonly in the stomach (60%), jejunum and ileum (30%), duodenum (5%), colon rectum (<5%), and rarely in other...

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References and Further Reading

  1. Corless, C. L., Barnett, C. M., & Heinrich, M. C. (2011). Gastrointestinal stromal tumours: Origin and molecular oncology. Nature Reviews Cancer, 11(12), 865–878. doi:10.1038/nr3143.PubMedGoogle Scholar
  2. Gouveia, A. M., Pimenta, A. P., Capelinha, A. F., de la Cruz, D., Silva, P., & Lopes, J. M. (2008). Surgical margin status and prognosis of gastrointestinal stromal tumor. World Journal of Surgery, 32, 2375–2382.PubMedCrossRefGoogle Scholar
  3. Heinrich, M., Corless, C. L., Demetri, G. D., von Mehren, M., Joensuu, H., McGreevey, L. S., Chen, C. J., Van den Abbeele, A. D., Druker, B. J., Roberts, P. J., Singer, S., Fletcher, C. D., Silberman, S., Dimitrijevic, S., & Fletcher, J. A. (2003). Kinase mutations and imatinib response in patients with metastatic gastrointestinal tumor. Journal of Clinical Oncology, 21, 4342–4349.PubMedCrossRefGoogle Scholar
  4. Martinho, O., Gouveia, A., Silva, P., Pimenta, A., Reis, R. M., & Lopes, J. M. (2009). Loss of RKIP expression is associated with poor survival in GISTs. Virchows Archives, 455, 277–284.CrossRefGoogle Scholar
  5. Miettinnen, M., Fetsch, J. F., Sobin, L. H., & Lasota, J. (2006). Gastrointestinal stromal tumors in patients with neurofibromatosis: a clinicopathologic and molecular genetic study of 45 cases. American Journal of Surgical Pathology, 30, 90–96.CrossRefGoogle Scholar
  6. Miettinnen, M., Fletcher, C. D. M., Kindblom, L.-G., & Tsui, W. M. S. (2010). Mesenchymal tumours of the oesophagus, stomach, small intestine, colon and rectum. In F. T. Bosman, F. Carneiro, R. H. Hruban, & N. E. Theise (Eds.), WHO classification of tumours of the digestive system. Lyon: International Agency for Research on Cancer (IARC) (pp. 35–36, 74–76,115–116, and 181–182, respectively).Google Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Faculty of Medicine of the University of Porto and Institute of Molecular Pathology and Immunology of the University of PortoPortoPortugal