A novel risk score to predict early and late recurrence in solitary fibrous tumour

Abstract

Aims: Solitary fibrous tumours (SFTs) are rare mesenchymal neoplasms with recurrence rates of 10–30%. Current risk stratification systems for extrameningeal SFTs are based on cohorts with limited follow-up and are not suitable for prediction of late recurrences. In this study we aimed to develop a prognostic model accounting for both early and late recurrences using a relatively large patient cohort with long-term follow-up. Methods and results: Clinicopathological factors were analysed in a cohort of 100 extrameningeal, STAT6-positive SFTs. Median follow-up for overall survival (OS) and recurrence-free interval (RFi) were 121 and 84 months, respectively. Disease relapse occurred in 31% of patients and median time to recurrence was 63 months. In univariate analysis mitotic count, necrosis, male gender and presence of severe atypia and pleomorphism were associated with inferior RFi. Mitotic count, necrosis and male gender were independent predictors of recurrence in multivariate analysis. Previously published risk models were also statistically associated with RFi in our cohort, but failed to reliably identify low-risk patients due to poor prediction of late recurrences. A novel risk score based on mitotic count, necrosis and gender was able to stratify patients into low-, intermediate- and high-risk groups for both early and late recurrences. Conclusions: In this cohort of patients with extrameningeal SFT and long-term follow-up mitotic count, necrosis and gender were independent prognostic markers of recurrence. We propose a novel risk score based on these factors and accounting for late recurrences, which should be validated in external cohorts with sufficient follow-up time.