A critical analysis of treatment strategies in desmoid tumours: a review of a series of 106 cases

Abstract 

Background

The management of desmoid tumours, previously based on strategies employed for sarcomas, should be reassessed, given the morbidity of interventions used in their treatment.

Methods

Long-term follow-up (median 123months) of a series of 106 treated patients with 69 primary and 37 recurrent desmoids, in order to study natural history and outcome.

Results

Desmoids typically evolved actively over a median period of 3years, and stabilised thereafter. Recurrences or progression most commonly occurred between 14 and 17months. Risk factors for recurrence were presentation (primary vs. recurrent), gender, tumour location and resection margins. However, survival was independent from these factors, with equivalent survival whether resection had been performed or not. Tumour control and functional outcome depended on location and presentation. Functional impairment was proportional to number of operations and whether patients had received radiotherapy. Recurrences were observed in 12/23 patients after radiotherapy.

Conclusion

Desmoids are relatively indolent tumours needing different approaches than sarcomas. Direct surgery is advisable only in primary lower trunk wall/girdle locations. Wait-and-see and medical treatment is preferable in other types of presentations.

Keywords: Desmoid tumours, Aggressive fibromatosis, Treatment, Surgery, Radiotherapy, Benign disease, Functional impairment, Medical treatment