Sarcoma

Question 1

First Line Systemic Therapy options (preferred and other treatment options) for Tenosynovial Giant Cell Tumor/Pigmented Villonodular Synovitis & associated mutation

Answer 1

First Line/Preferred: Pexidartinib (CSF1 Inhibitor)
Additional Treatment options: Imatinib, Nilotinib
Asssociated mutation: CSF1

Question 2

third-line agent for patients with metastatic or unresectable GIST

Answer 2

Regorafenib
160 mg of oral regorafenib daily on days 1-21 of a 28-day cycle

Question 3

GIST risk categories and indication for adjuvant treatment

Answer 3

Treatment:
Imatinib 400 mg daily for 3 years
Indication: rule of 5s
- 5 cm + mitotic rate > 5/50
- 10 cm with any mitotic rate
- ckit exon 9 mutation

Question 4

gist tx with PDFRGA D842V mutation

Answer 4

Avapritinib