Sarcoma

Question 1

What is the first line tx for GIST for locally advanced/metastatic disease assuming the exon 18 D842V mutation is not present?

Answer 1

Imatinib

Question 2

What is the preferred tx for locally advanced or metastatic GIST that harbors PDGFRA Exon 18 D842V? What is the second line agent for this mutation?

Answer 2

Avapritinib
2nd line-Dasatinib

Question 3

What is the 2nd line tx for GIST for locally advanced/metastatic dx with the exception of the exon 18 D842V mutation? What is the 3rd/4th line tx?

Answer 3

Sunitinib, if they are intolerant to this then you can consider ripretinib 150mg daily. 3rd line-Regorafenib. 4th line-Ripretinib (if not used before).

Question 4

What do you do for SDH mutated GIST that is locally advanced or metastatic?

Answer 4

Sunitinib, Regorafenib, Pazopanib, Imatinib/binimetinib (Cat 2B). NTRK-entrectinib, repotrectinib, larotrectinib. BRAF V600E-dabrafenib/trametinib.

Question 5

c-KIT exon 11 mutations are more common in what type of GIST tumor compared to c-KIT 9 mutations?

Answer 5

c-KIT exon 11-Gastric
c-KIT exon 9-small bowel