Advances in the multimodal management of central nervous system solitary fibrous tumors.
Solitary fibrous tumors (SFTs) of the central nervous system (CNS) are rare mesenchymal fibroblastic neoplasms. Although surgical resection remains the primary treatment, achieving gross-total resection is often challenging due to the frequent proximity of these tumors to critical neurovascular structures. Consequently, postoperative recurrence rates can be as high as 43%, and there is a notable risk of metastasis, underscoring the need for effective multimodal management.This review synthesizes current evidence on therapeutic strategies for CNS SFTs.Maximal safe surgical resection remains the cornerstone of local control. For patients undergoing subtotal resection, or those with high-risk pathological features (e.g., WHO grade II/III, high mitotic count, or necrosis), postoperative radiotherapy can significantly improve local control rates.Regarding systemic therapy, anti-angiogenic agents (e.g., pazopanib) have demonstrated superior disease control compared to conventional chemotherapy in patients with advanced, recurrent, or metastatic non-dedifferentiated SFT. Cytotoxic chemotherapy (e.g., doxorubicin) is now largely reserved for refractory or dedifferentiated subtypes. Immunotherapy (e.g., PD-1/PD-L1 inhibitors) has shown limited but promising activity in select patients, with PRAME emerging as a potential novel immunotherapeutic target.Optimal patient management requires a multidisciplinary team (MDT) approach involving specialists from neurosurgery, radiation oncology, and medical oncology.Treatment decisions should be individualized based on the extent of resection, tumor grade, location, and patient-specific factors. Lifelong, regular MRI surveillance is crucial for early detection of recurrence.Future directions should focus on conducting prospective clinical trials to generate higher-level evidence, further elucidating the NAB2-STAT6 fusion oncogene pathway, and developing rational combination therapies to improve outcomes for patients with this challenging tumor.