. 2019 Jul 5;18(3):2179–2191. doi: 10.3892/ol.2019.10575

Table IV.

First and second line options for the predominant histological subtypes of STS of the upper extremities.

Sarcoma type	First line	Second and further lines	Drugs under investigation
UPS	Doxorubicin ± Ifosfamide^a	Gemcitabine-Docetaxel^b; Ifosfamide^c; Trabectedin^d; Pazopanib^e	Pembrolizumab^f
SS	Doxorubicin ± Ifosfamide^a	Ifosfamide^c; Trabectedin^d; Pazopanib^e	Tazemetostat
ES	Doxorubicin ± Ifosfamide^a	Gemcitabine-Docetaxel^b; Pazopanib^e; Trabectedin^d	Tazemetostat
CCS	–	–	Caffeine-potentiated doxorubicin; Sorafenib; Sunitinib; Tinvatinib

Doxorubicin 60–75 mg/m² ± ifosfamide up to 9 g/m² days 1–3 every 21 days until disease progression or unacceptable toxicity (79).

Gemcitabine 900 mg/m² on days 1 and 8 + Docetaxel 100 mg/m² from day 8 every 21 days until disease progression or unacceptable toxicity (83).

Ifosfamide 14 g/m², continuous infusion for 6 days every 21 days with MESNA until disease progression or unacceptable toxicity (89).

Pazopanib 800 mg/day until disease progression or unacceptable toxicity (82).

Trabectedin 1.5 mg/m² in 24-h continuous infusion every 21 days until disease progression or unacceptable toxicity (81). STS, soft tissue sarcomas; UPS, undifferentiated pleomorphic sarcoma; SS, synovial sarcoma; ES, epithelioid sarcoma; CCS, clear cell sarcoma.