sarcoma

Question 1

synovial sarcoma systemic regimens

Answer 1

Adriamycin, ifosfamide, mesna (AIM)
High dose ifosfamide

Question 2

SOC for osteogenic osteosarcoma

Answer 2

Neoadjuvant cisplatin, doxorubicin, and high dose methotrexate (MAP)
Surgery
Additional adjuvant MAP chemotherapy (systemic disease that requires intense systemic chemotherapy)

Question 3

First line chemotherapy for osteogenic osteosarcoma

Answer 3

Neoadjuvant cisplatin, doxorubicin, and high dose methotrexate (MAP)

Question 4

Management of low-grade localized chondrosarcoma

Answer 4

Upfront surgical resection
Given low grade, observation with serial imaging

Question 5

Giant cell tumor management

Answer 5

Upfront surgery
*no role for radiation or chemotherapy

Question 6

Angiosarcoma of the breast management

Answer 6

mastectomy alone

Question 7

Preferred second line for GIST

Answer 7

Repritinib (INVICTUS)

Question 8

Second line for dedifferentiated liposarcoma

Answer 8

Eribulin

Question 9

management of recurrent desmoid tumor

Answer 9

sorafenib

Question 10

Management of recurrent giant cell tumor

Answer 10

Denosumab

Question 11

Firstline for GIST harboring PDGFRA exon 18 mutation

Answer 11

avapritinib

Question 12

Trabectedin SE to know

Answer 12

Rhabdomyolysis

Question 13

First line for metastatic osteosarcoma

Answer 13

IF >40, cisplatin/doxorubicin without high dose methotrexate

Question 14

Management of desmoid fibromatosis in pregnant patient

Answer 14

Monitor (DF that develops within 6 months of pregnancy may regress without intervention, so watchful waiting appropriate)

Question 15

GIST management with c-kit exon 9 mutation

Answer 15

higher starting dose of imatinib (400 mg BID) (higher mitotic rate and increased recurrence risk)

Question 16

Preferred second line for GIST

Answer 16

ripretinib

Question 17

first line for epithelial sarcoma

Answer 17

Tazemotostat

Question 18

Management of retroperitoneal leiomyosarcoma

Answer 18

surgery alone

Question 19

Preferred second line for KS

Answer 19

Taxol

Question 20

First line systemic therapy for metastatic soft tissue sarcoma

Answer 20

Doxorubicin