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Histology-specific targeted or immunotherapy treatment

Source: Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: February 21, 2025 · Captured 2026-09-09.

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Context: Treatment of Stage IV Soft Tissue Sarcoma / Treatment Options for Stage IV Soft Tissue Sarcoma

Although doxorubicin alone has traditionally been considered the standard when comparing new drugs or regimens in the context of phase III clinical trials, some sarcoma subtypes have shown higher sensitivity to specific agents. Table 9 provides examples of specific agents that can be used as frontline treatment for specific subtypes.

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Table 9

Table 9. Sarcoma Subtypes With Higher Sensitivity to Specific Agents
Sarcoma Subtype	Specific Agent
ALK = anaplastic lymphoma kinase; mTOR = mammalian target of rapamycin; PEComa = perivascular epithelioid cell tumor; TKI = tyrosine kinase inhibitor.
Alveolar soft-part sarcoma	TKIs, including sunitinib [16] and pazopanib [17]
Alveolar soft-part sarcoma	Immunotherapy, including atezolizumab [18] and pembrolizumab [19]
Angiosarcoma	Taxanes [20]
Dermatofibrosarcoma protuberans	Imatinib [21,22]
Inflammatory myofibroblastic tumor	ALK inhibitors [23]
PEComa	mTOR inhibitors [24], including nab-sirolimus [25]
NTRK-fusion-associated sarcomas	Larotrectinib [26]
Epithelioid sarcoma	Tazemetostat [27]
Desmoid tumors	Gamma secretase inhibitors, including nirogacestat [28]
Desmoid tumors	TKIs, including sorafenib [29]

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Treatment of desmoid tumors →

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