Sarcoma

Question 1

GIST 1st and 2nd line treatment

GIST KIT exons and responses to imatinib

Answer 1

KIT/PDGFR TKIs
1st line: Imatinib (400 mg QD  800 mg QD)
2nd line: Sunitinib
3rd line: Reografenib
4th line: Ripretinib

KIT/PDGFR specific mutation subsets
KIT Exon 11 = good response to imatinib (11 is heaven)
KIT Exon 9 = poor response to imatinib (9 is not fine). Try 800 mg.
KIT Exon 17 = resistance to imatinib/sunitinib (17 is mean)
PDGFR D824V = resistance to imatinib (use avapritinib)

Question 2

Treatment for symptomatic or visceral Kaposi Sarcoma

Answer 2

Doxorubicin or paclitaxel monotherapy

Relapsed/refractory = pomalidomide

Question 3

Osteosarcoma and Ewings sarcoma – chemo is given. Which ones?

Answer 3

Osteosarcoma: Neoadjuvant/adjuvant chemo with Doxorubicin/cisplatin +/- MTX (adjuvant RT if + margins)

Ewings: Neoadj chemo –> surgery –> adj chemo with VDC/IE (and RT if + margins)
VDC = vincristine, doxorubicin, cyclophosphamide
IE = ifosfamide/etoposide

Question 4

Treatment for non-localized desmoid tumors

Answer 4

TKI = sorafinib
or
Chemo = Doxil or doxorubicin +/- decarbazine

Question 5

Chondrosarcoma Treatment

Answer 5

Surgical resection

NO role for chemo or RT

Question 6

When do you give imatinib for adjuvant tx in GIST?

Answer 6

High risk = size > 5 cm, high mitotic rate, or tumor rupture

Give imatinib x 3 years

Question 7

Ewing Sarcoma - after surgery, what is given?

Answer 7

Adjuvant chemo (even if already got neoadjuvant chemo) 
If + margins --> adjuvant RT