LAUSR

Simple Summary In the latest WHO classification, solitary fibrous tumors (SFTs) are now subdivided into benign SFT (intermediate category (locally aggressive)), SFT NOS (intermediate category (rarely metastasizing)), and malignant SFT. Thanks to recent progress in molecular characterization, the identification of the NAB2–STAT6 fusion oncogene has emerged as a specific cytogenetic hallmark for SFT. Despite these recent advances in classification and understanding of the molecular pathophysiology of SFT, there are no consensus clinical guidelines regarding systemic treatment. Several new therapeutic options are of interest in this subtype of sarcoma considered as refractory to classical chemotherapy. In case of advanced disease, antiangiogenic therapy might be viewed as the best therapeutic option. Abstract SFT is an ultrarare mesenchymal ubiquitous tumor, with an incidence rate <1 case/million people/year. The fifth WHO classification published in April 2020 subdivided SFT into three categories: benign (locally aggressive), NOS (rarely metastasizing), and malignant. Recurrence can occur in up to 10–40% of localized SFTs, and several risk stratification models have been proposed to predict the individual risk of metastatic relapse. The Demicco model is the most widely used and is based on age at presentation, tumor size, and mitotic count. Total en bloc resection is the standard treatment of patients with a localized SFT; in case of advanced disease, the clinical efficacy of conventional chemotherapy remains poor. In this review, we discuss new insights into the biology and the treatment of patients with SFT. NAB2–STAT6 oncogenic fusion, which is the pathognomonic hallmark of SFT, is supposedly involved in the overexpression of vascular endothelial growth factor (VEGF). These specific biological features encouraged the successful assessment of antiangiogenic drugs. Overall, antiangiogenic therapies showed a significant activity toward SFT in the advanced/metastatic setting. Nevertheless, these promising results warrant additional investigation to be validated, including randomized phase III trials and biological translational analysis, to understand and predict mechanisms of efficacy and resistance. While the therapeutic potential of immunotherapy remains elusive, the use of antiangiogenics as first-line treatment should be considered.