Systemic Therapy for Solitary Fibrous Tumor.

Area of Science:

• Oncology
• Sarcoma Research
• Medical Therapeutics

Background:

• Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm classified as a soft tissue sarcoma (STS).
• While historically considered indolent, SFTs have a significant risk of recurrence or metastasis (up to 50%).
• Management of advanced or metastatic SFT presents challenges, necessitating effective systemic therapies.

Purpose of the Study:

• To review current systemic therapeutic strategies for solitary fibrous tumors (SFTs).
• To highlight the role of antiangiogenic therapy in advanced SFT management.
• To discuss emerging and future therapeutic approaches for SFT.

Main Methods:

• Literature review of current systemic therapeutic strategies for SFT.
• Summary of data on antiangiogenic agents, chemotherapy, and targeted therapies.
• Discussion of evolving treatment concepts, biomarkers, and future agents.

Main Results:

• Complete surgical resection is the primary treatment for localized SFT.
• Antiangiogenic agents show promise, especially for non-dedifferentiated SFT, and are increasingly used as first-line options.
• Traditional chemotherapy has limited efficacy; combinations with dacarbazine or trabectedin are being explored.

Conclusions:

• Antiangiogenic therapy is the current standard backbone for advanced SFT.
• Chemotherapy, molecularly targeted, and immunomodulatory approaches are emerging options requiring further validation.
• Evolving concepts include treatment sequencing, predictive biomarkers, and novel therapeutic combinations.