James Cassidy, MD, PhD, Chief Medical Officer at SpringWorks Therapeutics, gives an overview of Desmoid tumors.
As Dr. Cassidy explains, Desmoid tumors are tumors that can grow anywhere on the body into surrounding healthy tissues, including joints, muscle, and viscera. These rare tumors can cause significant pain, internal bleeding, and reduced mobility. Although these tumors do not metastasize, they can be locally aggressive. Recurrence is common for this condition and each recurrence, the tumors tend to be more aggressive.
Adults Versus Children
In the second video clip above, Dr. Cassidy notes that in adults, the average age of onset is 30-40 years and it predominantly affects women. It can also occur in children but no sex difference is observed. Since recurrence is common in Desmoid tumors and children start treatment at an earlier age, there is higher chance of having multiple rounds of recurrence in their lives. With each recurrence, there is also the increased likelihood the tumors will be more aggressive.
Currently, there is no systemic therapy approved for Desmoid tumors, but nirogacestat is currently being evaluated in a global phase 3, double-blind, placebo-controlled clinical trial (the DeFi trial) in adults, as well as in a pediatric study.
For more information about Desmoid and other rare tumors, visit checkrare.com/cancers/