Soft Tissue sarcoma

Question 1

Soft Tissue Sarcoma Subtypes with Non-Specific Histologies

Combination regimens

Answer 1

• AD (doxorubicin, dacarbazine)
• AIM (doxorubicin, ifosfamide, mesna)
• MAID (mesna, doxorubicin, ifosfamide, dacarbazine)
• Ifosfamide, epirubicin, mesna
• Gemcitabine and docetaxel
• Gemcitabine and vinorelbinef
• Gemcitabine and dacarbazine

Question 2

Soft Tissue Sarcoma Subtypes with Non-Specific Histologies

Single agents

Answer 2

• Doxorubicin
• Ifosfamide
• Epirubicin
• Gemcitabine
• Dacarbazine
• Liposomal doxorubicin
• Temozolomide
• Vinorelbinef
• Pazopanib
• Eribulin
• Trabectedin

Question 3

GIST

Answer 3

• Imatinib
• Sunitinib
• Regorafenib

Disease progression after imatinib, sunitinib, and regorafenib
• Sorafenib
• Nilotinib
• Dasatinib(for patients with D842V mutation)
• Pazopanib

Question 4

Desmoid Tumors (Aggressive fibromatosis)

Answer 4

• Sulindac or other non-steroidal anti-inflammatory drugs (NSAIDs) including celecoxib
• Tamoxifen ± Sulindac
• Toremifene
• Methotrexate and vinblastine
• Low-dose interferon
• Doxorubicin-based regimens
• Imatinib
• Sorafenib
• Methotrexate and vinorelbine
• Liposomal doxorubicin

Question 5

Non-Pleomorphic Rhabdomyosarcoma Combination regimens

Answer 5

• Vincristine, dactinomycin,
cyclophosphamide
• Vincristine, doxorubicin, cyclophosphamide
• Vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide
• Vincristine, doxorubicin, ifosfamide
• Cyclophosphamide and topotecan
• Ifosfamide and doxorubicin
• Ifosfamide and etoposide
• Irinotecan and vincristine
• Vincristine and dactinomycin
• Carboplatin and etoposide
• Vinorelbinef and low-dose cyclophosphamide
• Vincristine, irinotecan,temozolomide

Question 6

Non-Pleomorphic Rhabdomyosarcoma

Single agents

Answer 6

• Doxorubicin
• Irinotecan
• Topotecan
• Vinorelbine
• High-dose methotrexate
• Trabectedin

Question 7

Pigmented Villonodular Synovitis/Tenosynovial Giant Cell Tumor (PVNS/TGCT)

Answer 7

• Imatinib

Question 8

Angiosarcoma

Answer 8

• Paclitaxel
• Docetaxel
• Vinorelbine
• Sorafenib
• Sunitinib
• Bevacizumab
• All other systemic therapy options as per Soft Tissue Sarcoma Subtypes with Non-Specific Histologies

Question 9

Solitary Fibrous Tumor/Hemangiopericytoma

Answer 9

• Bevacizumab and temozolomide

* Sunitinib

Question 10

Alveolar Soft Part Sarcoma (ASPS)

Answer 10

• Sunitinib (category 2B)

Question 11

PEComa, Recurrent Angiomyolipoma, Lymphangioleiomyomatosis

Answer 11

• Sirolimus
• Everolimus
• Temsirolimus

Question 12

Inflammatory Myofibroblastic Tumor (IMT) with Anaplastic Lymphoma Kinase (ALK) Translocation

Answer 12

• Crizotinib

* Ceritinib

Question 13

Well-differentiated/Dedifferentiated Liposarcoma (WD-DDLS) for Retroperitoneal Sarcomas

Answer 13

• Palbociclib