Evaluation of outcomes in the management of primary sporadic desmoid-type fibromatosis at a specialist soft tissue sarcoma unit

Abstract

Abstract Background Desmoids are rare fibroblastic tumours whose treatment in any individual case presents a persistent challenge. We endeavoured to evaluate various clinicopathological factors contributing to tumour behaviour. Methods This is a retrospective review of 95 primary truncal sporadic fibromatosis managed between 2011 and 2020. We studied progression rate for wait and watch (WW) and recurrence rate for the surgically treated group as adverse events. Relevant event-free survivals and potential risk factors were analysed over a median follow-up of 27 months. Results 66 patients (69.5%) received watchful surveillance and 28 upfront surgery. 2-year progression-free survival in WW group (88.9%) was higher than RFS in the surgical group (77.1%) p = 0.02. Adverse event rate compared favourably, 28.8% in WW and 28.6% in surgical group. At final follow-up, rate of stable disease for WW was 47%, and the regression rate was 24.2%. On Cox regression analysis, meantime to progress was 14 ± 2.0 months, with larger tumour size as a significant prognostic indicator (p = 0.05). Surgical group's mean time to recurrence was 13.8 ± 2.76 months, with tumour location a significant contributing factor (p = 0.05). Conclusions This study confirms to the safety of both treatment approaches. Adverse event rates remained comparable, but event-free survival was longer for the watchful surveillance group.

Keywords

• Sporadic desmoid fibromatosis
• Watchful waiting
• Surgical resection of desmoid
• Active surveillance
• Prognostic factors
• Event-free survival