Gastrointestinal stromal tumor (GIST)

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Introduction:

  • Mesenchymal in origin
  • Cell of origin: Interstitial cell of Cajal (pacemaker of intestine)
  • MC site: Stomach (50%) > Ileum [Reference: 27th Bailey]
  • MC presentation: Bleeding
  • Criteria used to determine low/ high risk GIST: Fletcher criteria.

Investigation:

  • IOC for GIST: CECT
  • IOC for recurrent GIST: PET scan.

Markers:

  • Most sensitive: CD117 (c-KIT)
  • Most specific: Dog1.

Management:

  • Primary management is surgery with 2 cm clear margin
  • Malignant/ metastatic GIST: Add Imatinib (Tyrosine kinase inhibitor)
  • DOC of Imatinib resistant GIST: Sunitinib.

Carney’s triad:

  1. GIST
  2. Pulmonary chondroma
  3. Extra-adrenal paraganglioma.

Note: GISTs seen in Carney’s triad show primary resistance to Imatinib.

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