Extra-abdominal desmoid tumors

Desmoid tumours are aggressive fibromatoses with a high recurrence rate. Their management is multidisciplinary. Surgery has given way to active surveillance as a first-line treatment. Stabilisation and spontaneous regression are reported in 50% of cases.Surgery is still appropriate for progressive tumours that can be removed without vital or functional damage. Radiotherapy is indicated alone if surgery is not possible or postoperatively if removal is insufficient. Medical treatment is an alternative for advanced tumours or in case of recurrence.