Gastrointestinal Stromal Tumor (GIST) - A Rev...

Gastrointestinal Stromal Tumor (GIST) - A Review

Author Name : Rahul Dasgupta

ABSTRACT

Gastrointestinal stoma tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract and most common malignancy of the small intestine. Special interest in the role of tyrosine kinase in its regulation has been the target by different drug research. Mutation in c-kit exons 9, 11, 13, 17 and PDGFRA mutation in exons12,14,18 are responsible for activation of gene signaling system resulting in uncontrolled phosphorylation and tissue growth.  Histological, GISTs look like spindle cell tumors most of the time but they can be epithelia or mixed type. The median size of GISTs varies from 2.7 cm to 8.9 cm. Diagnosis of GISTs relies heavily on KIT/CD117 immunohistochemical staining, which can detect most GISTs except for a few 3.5% to 5.5% that harbors PDGFRA mutation.Newer staining against PKC theta and DOG-1 genes showed promising results but are not readily available. . Surgery still remains the first-line treatment for GISTs. The advancement of molecular biology has revolutionized the availability of newer drugs, Imatinib and Sunitinib. Together with its advancement is the occurrence of Imatinib/Sunitinib drug resistance. With this, newer monoclonal antibody drugs are being developed and are undergoing clinical trials to hopefully improve survival in patients with GISTs.

Key Words -Gastrointestinal Stromal Tumor; Mesenchymal Tumor of Gastrointestinal Tract; Gastrointestinal Subepithelial Tumors; Management of Gastrointestinal Stromal Tumor; Familial Gastrointestinal Stromal Tumor; Risk Stratification