Understanding When to Choose Systemic Treatment for Desmoid Tumors

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EP: 1.Understanding When to Choose Systemic Treatment for Desmoid Tumors

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Mark Agulnik, MD, professor of Clinical Medicine, Vice Chair for Faculty Development, Department of Medicine, and Section Chief of Sarcomas & Melanoma at the Keck School of Medicine of USC, discusses the intricacies of desmoid tumors that can be recognized before starting treatment for patients.

Desmoid tumors are complex conditions with a pattern of growth and regression, necessitating careful monitoring of their longitudinal progression, according to Agulnik. Initial treatment typically does not start unless there's an urgent medical need, focusing instead on evaluating the natural history of the disease.

Treatment is considered if the tumor causes significant pain, impairs function, or negatively impacts the patients’ quality of life. Standard therapy now favors drug treatments over surgical resection due to the high recurrence risk associated with surgery. Agulnik says this shift reflects improved understanding of the disease's behavior, leading to more patient-beneficial systemic therapies.

TRANSCRIPTION:

0:10 | Desmoid tumors are very complex. There's a very unique process to this condition. There is a ‘wax and wane’ type of quality, and so patients can have growth of the tumor, and then they can have subsequent regression of the tumor. So, one needs to be very aware of what's happening. They have to understand the longitudinal process of the disease, and also, they have to understand what are the inherent needs at the time that the patients are seen.

0:37 | If a patient is seen for the first time, there likely is not a need to start treatment unless there is an impending medical emergency, but for the most part, these patients need to be evaluated, and then a longitudinal evaluation needs to be done to understand the natural history of the disease. If the disease or the tumor grows over time or is causing significant problems for the patient with respect to pain, disruption of ability to function [or] their activities of daily living, or affecting quality of life, then it is reasonable to start patients on a therapy. Currently, the standard first-line therapies would be drug therapies for most patients. Many years ago, the standard treatments would have been for surgical resection. But that has changed over time because we have a better understanding that these diseases do come back after surgical resections for many patients, and therefore, if we could take a path to systemic therapy or drug therapy, that is in the patient's best interest.