Surgery

Surgical resection should be used judiciously in patients with desmoid tumors because spontaneous regression can occur in up to 20% of cases. Surgical resection is recommended when tumor enlargement threatens the airway or when symptoms such as pain are persistent. A watch-and-wait strategy is otherwise preferred.

If surgery is chosen, the intent is to achieve clear margins. However, a retrospective review of children who underwent surgery for desmoid-type fibromatosis at St. Jude Children’s Research Hospital (SJCRH) reported no correlation between surgical margins and risk of recurrence.[19] In this study, 10 of 39 patients experienced a recurrence after surgery, with a median interval time of 2.5 years.