Dr. Riedel on the Challenges of Diagnosing and Treating Patients With Desmoid Tumors

Richard F. Riedel, MD, associate professor of medicine, Department of Medicine, Duke University School of Medicine, member, Duke Cancer Institute, medical oncologist, Duke Cancer Center, Duke Health, discusses the challenges of diagnosing and treating patients with desmoid tumors.

Desmoid tumors are bland in appearance and can occur spontaneously anywhere in the body, Riedel says. The tumors are often indolent and cannot metastasize; however, they can grow to be locally invasive and aggressive. Although uncommon, patients who seek medical attention and undergo a biopsy could be misdiagnosed with low-grade sarcoma, for example. As such, pathologic confirmation of the diagnosis with immunohistochemistry is recommended.

Research is ongoing to evaluate gamma-secretase inhibitors as potential therapeutic options in patients with desmoid tumors, Riedel explains. These agents inhibit the Notch pathway, which may prevent tumor growth in these patients. Nirogacestat, a gamma-secretase inhibitor, is being evaluated in the phase 3 DeFi trial (NCT03785964) in patients with desmoid tumors or aggressive fibromatosis. Other agents, such as AL102, are being investigated. Finally, combination therapies could emerge as options to improve upon single-agent systemic and chemotherapeutic regimens, Riedel adds.

Notably, to mitigate some of the diagnostic and therapeutic challenges experienced in the field, education in community practices is needed, Riedel says. It should be underscored in clinical practice that surgical resection may not be the optimal treatment approach because desmoid tumors may disappear with observation and surgery can cause unnecessary complications. Additionally, patients should be treated by a multidisciplinary care team that includes medical, surgical, and, in some cases, radiation oncologists, to individualize patient care, concludes Riedel.

Funding from SpringWorks Therapeutics/Content Developed Independently by OncLive