Dr. Randall on Initial Considerations When Treating Patients With Desmoid Tumors

R. Lor Randall, MD, FACS, professor and The David Linn Endowed Chair for Orthopaedic Surgery and professor and chair of the Department of Orthopaedic Surgery, University of California Davis Health, discusses initial considerations when treating patients with desmoid tumors, a type of soft tissue sarcoma.

It is important to establish up front whether a patient with a diagnosed desmoid tumor harbors an APC mutation, which would put them at risk for Gardner syndrome, multiple desmoid fibromatosis, and polyps, Randall explains. Patients with Gardner syndrome need to be monitored aggressively because they tend to have multiple relapses at multiple sites because they have several desmoids. As such, genetic counseling should be strongly considered for patients with a solitary desmoid tumor, particularly if they have a family history of colon cancer, Randall says.

Additionally, it is critical to weigh the risks and benefits of treatment for patients with desmoid tumors to ensure the treatment doesn’t cause more harm to the patient than surveillance, Randall says. Notably, not all patients with a desmoid tumor require aggressive local treatment, which was standard of care historically. In the past, some patients would be disfigured from aggressive treatment with the goal of negative margins; however, their disease likely didn’t require such interventions.

This concept has been observed in patients with desmoid tumors of the chest wall or breast implant–associated desmoid tumors, Randall explains. Moreover, treating patients with desmoid tumors with aggressive surgery and radiation therapy can lead to disfigurement, identity issues, and pain. Therefore, it is critical to get insight from a provider who has substantial experience in managing desmoid tumors before making treatment decisions for patients, Randall adds.

Historically, patients with desmoid tumors who required treatment underwent surgery with or without radiation therapy. Although surgery still plays an important role in the management of desmoid tumors, it has become less prominent. As such, the guidelines now are to monitor patients when their tumor is not progressing. If they have progressive disease, several noncytotoxic and cytotoxic therapies can be considered, Randall concludes.

Funding from SpringWorks Therapeutics/Content Developed Independently by OncLive