GASTROINTESTINAL STROMAL TUMORS (GIST)
GASTROINTESTINAL STROMAL TUMORS (GIST)
- Most common mesenchymal tumor of the abdomen
- Commonly occur in stomach (mc site) and duodenum
- Arise from the interstitial cells of Cajal or pacemaker cells
- Carney triad (non hereditary syndrome of young females)
- Gastric GIST
- Paraganglioma
- Pulmonary chondroma
- Bleeding is the most common presentation (Endoscopy — appearance of cervix)
- Diagnosis of malignancy is by greater mitotic figures
- If mucosa is not ulcerated — biopsy on a biopsy is needed
- Immunohistochemistry is mandatory for diagnosis. C-kit gene amplification — tumor marker
- Size and mitotic index are the best predictors of metastasis
- Peritoneal and liver metastases are most common
- Lymphatic spread extremely rare
- 80% tumors respond to Imatinib(tyrosine kinase antagonist)
- Treatment of choice — Resection
Exam Important
Tyrosine kinase inhibitors imatinib and sunitinib are approved for the treatment of Gastrointestinal Stromal Tumors (GIST)
Most common mesenchymal tumor of the abdomen
Commonly occur in stomach (mc site) and duodenum
Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract.
Arise from the interstitial cells of Cajal or pacemaker cells
Size and mitotic index are the best predictors of metastasis
Tumors whose behavior is driven by mutations in the KIT gene (85%), PDGFRA gene (10%) or BRAF kinase (rare)
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