Desmoid fibromatosis: IR (sometimes) to the rescue for an atypical disease.

ABSTRACT

Desmoid fibromatosis is a rare locally aggressive soft tissue tumor that is characterized as benign as it cannot metastasize. It was managed until recently like sarcomas, i.e with radical surgical resection combined or not with radiotherapy. However, this approach was associated with a high rate of recurrence and significant morbidity. The management of this disease has progressively changed to a more conservative approach given the fact that desmoid fibromatosis may spontaneously stop to grow or even shrink in more than half of the cases. Should treatment be required, recent guidelines recommend choosing between systemic therapies, which include principally chemotherapy and tyrosine kinase inhibitors, and local treatments. And this is where the interventional radiologist may have an important role to treat the disease. Various ablation modalities have been reported in the literature to treat desmoid fibromatosis, notably high-intensity focused ultrasound and cryoablation. Results are promising and cryoablation is now mentioned in recent guidelines. The interventional radiologist should nevertheless apprehend the disease in its globality to understand the place of percutaneous treatments among the other therapeutic options. The goal of this review is therefore to present and discuss the role of interventional radiology (IR) in the management of DF.