GIST


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  • Clinical

    • age: 50-60. Young in Carney triad.
    • location: stomach, intestines
      • also omentum, peritoneum, retroperitoneum
      • rarely esophagus
    • can be benign or malignant
      • intestinal GIST more likely malignant
    • associations: NF1, familial GIST (ckit, PDGFRA mutation), Carney triad (gastric GIST, paraganglioma, pulmonary chondroma)
  • Histology

    • sheets, fascicles, whirls, palisades
    • spindle and epithelioid cells
    • may have perinuclear vacuoles
    • features of malignancy
      • size > 10 cm
      • mitosis >= 1 per 10 hpf
      • for stomach, both criteria makes it malignant
      • for intestine, any criteria makes it malignant
  • Stains

    • CD117+
    • PDGFRA+
  • Molecular

    • ckit
    • PDGFRA
  • Ddx

    • smooth muscle tumors
      • desmin+
      • CD117-
    • neural tumors
      • S100+
      • CD117-
    • fibromatosis