What Are Desmoid Tumors?

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06/28/23

Bernd Kasper, MD, PhD, University of Heidelberg, Mannheim Cancer Center, Mannheim, Germany, gives an overview of desmoid tumors and how to diagnose them.

As Dr. Kasper explains, desmoid tumors 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. These benign tumors are notoriously recurrent, especially with surgical removal, and each recurrence tends to be more aggressive than the previous tumor.

Currently, there is no approved targeted therapy for desmoid tumors. However, positive data from the DeFi trial, a phase 3 trial evaluating the efficacy, safety and tolerability of nirogacestat in adult patients with progressing desmoid tumors were recently presented as a late-breaking oral presentation during a Presidential Symposium at the European Society for Medical Oncology (ESMO) Congress 2022. Additionally, SpringWorks Therapeutics plans to submit a New Drug Application (NDA) to the FDA in the second half of 2022.

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