Desmoid tumor: A focus set on a challenging but understudied rare disease

Summary

This Editorial highlights current developments and new paradigms in the management of desmoid tumor (DT) patients as developed by the Desmoid Tumor Working Group. The article “Autophagy Inhibition Overcomes Sorafenib Resistance in S45F?Mutated Desmoid Tumors” is put into perspective regarding a new emerging drug, sorafenib, which is proven to be active in DT in a recent phase 3 trial.