Table 2 The potential role of targeted therapies in histological subtypes that are poorly responsive to chemotherapy [modified from [220]].
From: UK guidelines for the management of soft tissue sarcomas
STS subtype | Key characteristics | Drug |
|---|---|---|
Extraskeletal myxoid chondrosarcoma | EWSR1-NR3A3 gene fusion | |
Solitary fibrous tumour | Typical, i.e., low aggressiveness | Anti-angiogenic TKI [223] |
Desmoid fibromatosis | n/a | |
Alveolar soft part sarcoma | n/a | Anti-angiogenic TKI [225, 226], atezolizumab [227] or combination of TKI /checkpoint inhibitor [228, 229] |
Epithelioid haemangioendothelioma | n/a | mTOR inhibitors, e.g. sirolimus, TKIs [230] |
Inflammatory myofibroblastic tumour | n/a | ALK inhibitor [231] |
Epithelioid sarcoma | n/a | Tazemetostat [137] |
PEComa | n/a | |
Undifferentiated pleomorphic sarcoma | n/a | Immune checkpoint inhibitor [140] |
Angiosarcoma | Radiation-associated | Checkpoint inhibitor [140] |
