Abstrakt
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal (GI) tract. They are believed to originate from the interstitial cells of Cajal (ICCs) or from the precursors of ICCs.
Most GISTs show an activating mutation in either the c-kit or platelet-derived growth factor receptor alpha (PDGFRA) gene. Tumor size, mitotic rate, and anatomic location correlate with potential malignancy and recurrence rate.
A total of 12 patients were diagnosed to have GIST based on histology or immunohistochemistry of a biopsy or resection specimen obtained from the GI tract in the 2004-2009 period. The material was obtained using retrospective data collection.
Tumors classified as GISTs with low and very low risk of progression are associated with a very good prognosis, with virtually all patients surviving 5 years. In patients with high risk or progressive diseases, the prognosis of 5-year survival is much poorer.
The main therapeutic option is surgical removal of the tumor (resection or broad excision). Agents showing promise for patients with malignant forms of GISTs are tyrosine kinase receptor inhibitors.
Although imatinib is currently used as a first line treatment for all patients with metastatic or unresectable GISTs, it is likely that this treatment will change in the future based on the underlying mutational status.