Desmoid Fibromatosis: Management in an Era of Increasing Options

Abstract

Purpose of review: Desmoid fibromatosis (DF) is a locally aggressive clonal neoplasm with locally aggressive behavior and no metastatic potential. Historical treatment of DF has consisted primarily of up-front surgery when feasible. In recent years, recognition that DF can spontaneously stabilize or involute has allowed for many patients to be managed with watchful waiting rather than intervention. This review is intended to review recent developments in the treatment of DF.

Recent findings: Recent studies have demonstrated prospectively that patients with DF often have improvement in their lesions without intervention, enabling an initial period of surveillance as a standard option for patients with mild symptoms. Given the lengthening list of effective systemic treatments, including sorafenib, pazopanib, and experimental agents, there has been a less reliance on local therapies for those patients who require treatment. For patients with DF that require treatment, there is a growing list of options that includes radiation therapy (RT), percutaneous ablation, and a growing list of systemic agents with favorable toxicity profiles.

Keywords: Aggressive fibromatosis; Radiation therapy; Sorafenib; Systemic therapy.