Intracranial Solitary Fibrous Tumour Management: A French Multicentre Retrospective Study

SIMPLE SUMMARY: Intracranial solitary fibrous tumours (iSFTs) are exceptional mesenchymal tumours with a high relapse rate. We aimed to analyse the clinical outcome at each stage of the disease. We carried out a multicentre retrospective study including 88 patients from 16 French centres. Gross tumour resection was found to be a factor for good prognosis and significantly reduced local recurrence without impacting overall survival. High-grade tumours were a factor for poorer PFS and LRFS. More than 40% of our patients experienced local recurrence and were mostly treated with surgery and radiotherapy. The first relapse is a turning point in iSFT evolution, with reduced recurrence latency over the course of the disease. The management of repeated recurrence and disseminated diseases is challenging; these situations should be treated, if feasible, with local techniques considering the poor efficiency of systemic treatments. ABSTRACT: Background: Intracranial solitary fibrous tumour (iSFT) is an exceptional mesenchymal tumour with high recurrence rates. We aimed to analyse the clinical outcomes of newly diagnosed and recurrent iSFTs. Methods: We carried out a French retrospective multicentre (n = 16) study of histologically proven iSFT cases. Univariate and multivariate Cox models were used to estimate the prognosis value of the age, location, size, WHO grade, and surgical extent on overall survival (OS), progression-free survival (PFS), and local recurrence-free survival (LRFS). Results: Eighty-eight patients were included with a median age of 54.5 years. New iSFT cases were treated with gross tumour resection (GTR) (n = 75) or subtotal resection (STR) (n = 9) and postoperative radiotherapy (PORT) (n = 32, 57%). The median follow-up time was 7 years. The median OS, PFS, and LRFS were 13 years, 7 years, and 7 years, respectively. Forty-two patients experienced recurrence. Extracranial metastasis occurred in 16 patients. Median OS and PFS after the first recurrence were 6 years and 15.4 months, respectively. A higher histological grade was a prognosis factor for PFS (p = 0.04) and LRFS (p = 0.03). GTR influenced LRFS (p = 0.03). Conclusion: GTR provided benefits as a first treatment for iSFTs. However, approximately 40% of patients experienced relapse, which remains a challenging state.