Surgical Pathology Criteria

Gastrointestinal Stromal Tumor (GIST)

Multiple, Hereditary and/or Pediatric GIST – (all are frequently multiple; select individual disorders for details)
  Heredity Mean Age M/F Associated Lesions Mutations GIST Location Behavior
Familial AD 45 M&F Mast cell lesions, achalasia GL KIT /PDGFRA Small intestine Frequently aggressive
Carney-Stratakis AD 23 M&F Paraganglioma GL SDH, No KIT / PDGFRA Stomach epithelioid GIST mets but protracted, Paragang. aggressive
Carney Triad None <30 >95%F Lung chondroma, paraganglioma No KIT / PDGFRA or SDH Stomach epithelioid Mets but protracted course
NF1 AD 40-50 M&F Neurofibromatosis GL SDH, No KIT / PDGFRA Small intestine spindled As for usual
Sporadic SDHB Deficient (Pediatric Type) None <16, rarely also adults >90%F None No KIT / PDGFRA or SDH Stomach epithelioid Mets but protracted course, may go to nodes
Sporadic multiple None 60 M&F None As for usual Usually stomach Most are benign
 

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