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...
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References and Further Reading
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.
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.
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.
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.
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.
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).
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2017 Springer International Publishing AG
About this entry
Cite this entry
Lopes, J.M. (2017). Gastrointestinal Stromal Tumor. In: Carneiro, F., Chaves, P., Ensari, A. (eds) Pathology of the Gastrointestinal Tract. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-40560-5_1619
Download citation
DOI: https://doi.org/10.1007/978-3-319-40560-5_1619
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-40559-9
Online ISBN: 978-3-319-40560-5
eBook Packages: MedicineReference Module Medicine