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Gist noon conference
- 2. © 2016 Virginia Mason Medical Center 2 Objectives Gastrointestinal Stromal Tumor (GIST) • Pathophysiology • Discuss clinical presentation • Discuss diagnosis • Review illness script • Discuss treatment
- 3. © 2016 Virginia Mason Medical Center Pathophysiology and Other Info • Most common mesenchymal neoplasms of GI tract • CD117 expression – KIT gene • Histo • Subepithelial location • Majority (70%) are spindle cell-type • May originate from interstitial cells of Cajal 3
- 4. © 2016 Virginia Mason Medical Center Clinical presentation Affected Body Part Symptoms Gastrointestinal Tract Early satiety, bloating, abdominal discomfort, GI bleed, abdominal mass Endocrine Paraneoplastic (consumptive hypothyroidism, non-islet cell tumor hypoglycemia) Heme Anemia 4
- 5. © 2016 Virginia Mason Medical Center Diagnostic tests • CT with contrast – initial imaging • MRI preferred for GISTs in rectum or liver • Pre-operative biopsy is definitive, but not necessary 5
- 6. © 2016 Virginia Mason Medical Center Question 1 What percentage of GISTs initially present with bleeding? • A: 10% • B: 25% • C: 50% • D: 60% 6
- 7. © 2016 Virginia Mason Medical Center Staging/Prognosis • All GISTs regarded as having malignant potential • Increased metastatic risk: • Imaging: >5cm, lobulated heterogeneous enhancement • Intermediate Small bowel tumors • Decreased survival rate • Lymph node involvement • Tumor rupture 7
- 8. © 2016 Virginia Mason Medical Center Illness Scripts 8 Gastrointestinal Stromal Tumor Other GI Subepithelial tumors (Leimyoma, leiomyosarcoma) Pathophysiology CD117 (KIT) mutation Some other mutation Epidemiology 40+ F> M (peds) 1% of Primary GI tumors 7-15 per million per year M>F Time course Chronic Chronic Clinical presentation Early satiety, abdominal discomfort, GI bleed, abdominal mass, nausea, vomiting Early satiety, abdominal discomfort, GI bleed, abdominal mass, dysphagia, constipation, weight loss Diagnostics Labs: anemia CT/MRI: solid, smoothly contoured mass that enhances with IV contrast. Possible dumbbell shape Biopsy: CD117, CD 34, DOG-1 Labs: anemia CT: smoothly contoured, hypodense, adjacent to gut lumen; leiomyosarcomas tend to be large with internal necrosis. Possible dumbbell shape Biopsy: Desmin, PKC-theta, CD34 Therapeutics Imatinib, sunitinib, Surgery (localized, > 2cm) Surgery
- 9. © 2016 Virginia Mason Medical Center Question 2 What is the 5-year survival for GIST with neoadjuvant imatinib? • A: 20% • B: 50% C: 80% • D: 95% 9
Editor's Notes
- Title your presentation “Noon Conference” Prevents inadvertently giving away the case.
- Can arise anywhere in the GI tract although most commonly in the stomach or small bowel KIT – transmembrane tyrosine kinase; abnormally activated in this case A subset of GISTS lack KIT and have activating mutations for another related receptor tyrosine kinase, platelet-derived growth factor receptor alpha (PDGFRA)
- Typically present in middle-aged and older individuals (rare in patients under 40) Mostly sporadic, although there are some 5% familial conditions (KIT or PDGFRA mutations) that are predisposed to multiple gastric and small bowel GISTS w/ other features – hyperpigmented skin, dysphagic, paragangliomas or intestinal fibromatosis GISTs rare in children and usually part of a syndrome. Peds tend to have a succinate dehydrogenase (SDH) mutation
- Biopsy useful if you suspect mets or if you’re considering pre-op imatinib for a large or locally advanced tumor
- Ans: D
- Intermediate small bowel tumors >10cm w/ low mitotic rate and those <5cm w/ >5 mitoses per 50 hpf had >50% metastatic rate; gastric tumors have much lower risk Imaging: >5cm, lobulated heterogeneous enhancement, mesenteric fat infiltration, ulceration, regional LAD, exophytic growth pattern
- Other subepithelial tumors include malignant melanoma, schwannoma, malignant peripheral nerve sheath tumor, fibromatosis (desmoid tumor), inflammatory myofibroblastic tumor Leiomyosarcoma – typically in the stomach Leiomyoma – typically in esophagus, colon, rectum GIST F/u – CT every 3-6 mo for 3-5 years, then annually
- Ans: C