Systemic Therapy for Solitary Fibrous Tumor

Solitary fibrous tumor (SFT) is an exceedingly rare mesenchymal neoplasm classified as a soft tissue sarcoma (STS). While historically considered indolent, up to 50% of cases can recur or metastasize after long-term follow-up. Complete surgical resection constitutes the cornerstone of treatment for localized disease, while the management of locally advanced or metastatic SFT poses a significant clinical challenge underscoring the need for effective systemic therapies. Antiangiogenic agents have shown promising outcomes, particularly in non-dedifferentiated SFT, and are increasingly favored by some as first-line options. Regarding chemotherapy, traditional anthracycline-based STS regimens have demonstrated limited efficacy, shifting the current focus towards combinations with alkylating agents, such as dacarbazine, and other agents such as microtubule inhibitors, like trabectedin. This review summarizes current systemic therapeutic strategies for SFT, highlighting the central role of antiangiogenic therapy as the standard backbone for managing advanced disease. It also discusses current data on chemotherapy, as well as molecularly targeted and immunomodulatory approaches, which are emerging options that require further validation. In addition, we discuss evolving concepts in systemic treatment sequencing, predictive biomarkers, advanced therapeutic combinations under clinical evaluation, and potential future therapeutic agents and targets.