Dr. Lazar on the Current State of Diagnosis and Treatment in Desmoid Tumors

Alexander J. Lazar, MD, PhD, academic surgical pathologist, professor, Department of Pathology, Division of Pathology/Lab Medicine, director, Sarcoma and Melanoma Clinical Genomics Program, director, Selective (Soft Tissue) Pathology Fellowship Training Program, The University of Texas MD Anderson Cancer Center, discusses the current state of diagnosis and treatment in desmoid tumors.

Desmoid tumors are rare and look like reactive fibrosis or scarring, so they can be challenging to diagnose, Lazar explains. Unless desmoid tumors are included as potential diagnoses in the differential, clinicians who do not have a lot of experience with these tumors may diagnose them as being the reactive part of another process.

However, improvement in tools to differentiate spindle-cell neoplasms, misdiagnoses are not as common with desmoid tumors. Despite this, delays in diagnosis remain because desmoid tumors can be challenging to recognize as definitive tumors, Lazar adds.

Historically, patients with desmoid tumors were treated aggressively with surgery and sometimes radiation, Lazar explains. However, it’s been shown that patients can receive active surveillance and that early surgical interventions are not needed for all patients. Moreover, emerging oral or intravenous systemic therapies have the potential to further minimize the need for surgical interventions, which pose a morbidity and mortality risk to patients, Lazar says.

Although active surveillance is the standard therapy for patients diagnosed with desmoid tumors, determining which patients require more aggressive treatment remains an area of unmet need, Lazar says. Oftentimes, patients with stable disease are best suited for active surveillance, but patients with disease-related pain or dysfunction should receive surgery or radiation.

Moreover, emerging systemic therapies, such as sorafenib (Nexavar), gamma-secretase inhibitors, or beta-catenin inhibitors, may be indicated for patients in need of more aggressive therapies, Lazar concludes.

Funding from SpringWorks Therapeutics/Content Developed Independently by OncLive