Small Bowel And Gist Flashcards
Si tumor distribution
Adenoca 30-50% 15% has high msi
Net 25-30
Lumphoma 25
Life time risk of sb adenoca in syndromes
Fap
Lynch
Pjs
Fap 3-5% duo and periamp
Pjs- 1.7-13% RR-520
Lynch- 4% RR 100
Max serotonin production which gut net
Midgut net
Percent mets in appendiceal and ileal net
35 and 3%
Chance of mets per size of net
- NETs < 1 cm in diameter - 2% metastases
- NETs 1 to 2 cm - 50% metastases
- NETS > 2 cm - 80-90% metastases.
Small bowel nets multicentricitu
20-30%
Synchronous adenoca esp in large bowel in %
10-20%
Carcinoid syndrome in % of nets
10%
Mc heart ailmet in carcinoid
Rt heart
Pulm stenois
Tricuspid
Increased nterminal pro bmp and chrga prognosis
Worser than chrg a alome
Petct in net
68 ga dotatate-ioc in localisation
68cu dotatate
Tnm of net T
1- lp or sm and less than or eq to 1cm 2- muscprop or more than 1 cm 3- musc prop into subseros 4- visceral periton serosa and other organs N1- 12 nodes 2- large more than 2 cm mass or more than 12 nodes M1aliver B extrahep C both
RADIANT TRIAL
Everolimus in net
Netter trial
Lu 177 dotatate
Mc intestinal neoplasm in less thna 10 yrs
Si lymphoma distal ileum
Mets to si mc is
Melanoma
Gist sdh deficient
1Carney triad gist pulm chondroma extradrenal paragangliom
2Carney stratakis syndromegist and familial paragangliomas
3. Nf1
Wt for kit and pdgfra
10-15%
1.sdh def
2- nf1
3. Braf ras
Tyoes of gust
Spindle m/c
Epitheloid
And mixed
IHC FOR GIST
Cd117 ckit
Dog1
Cd34
Moleculygentic kit (m/c)and pdffra
Exons in gist
Kit- mc 11
Pdgfra mc 18 D842V
Mc site of exon 9
Kit si
Pdgfra stomach
Response rates to imatinib
Response rates to imatinib: • KIT exon 11 mutation - 90%. KIT exon 9 mutation - 50% - improves with 400 mg bd dose. Most PDGFRA mutations are associated with a response to imatinib, with the exception of D842V (Responds only to avapritinib)
Better prognoisis which site
Stomach
Surveillance feasible in gist?
Very small gastric gists les than 2 cm
Eithout high risk eus features like irregular border heterogenus echo pattern custic spaces echogenic foci
Adjuvant imatinib duration in gist
3 yrs for high risk gist - mitotic more than 5/50hpf Tumor rupture Sie more than five cm Nok gastric location In mets disease continue drug till progression
Fletchers criteria aka
Nih consensus criteria
To assess recurence rate
Miettinens criteria
Armed forces ip criteria risk stratification
Dose of adjuvant imatinib
400 mg od
800 mg if progressive
Or if exon 9 kit
Sunitinb dose
50 mg od 4 wks every 6 wks
Regorafenib dose
160 mg od 3 wks every 4 wks
Dasatinib when
D842 Vpdfra
