The Desmoid Tumor Research Foundation

May 2025

Evolving Concepts in the Management of Desmoid Tumors

Author Information

Sylvie Bonvalot 1, Aisha Miah 2, Bernd Kasper 3

1Department of Surgical Oncology, Institut Curie, 26 rue d'Ulm, Paris 75005, France. Electronic address: sylvie.bonvalot@curie.fr. 2Sarcoma Unit, The Royal Marsden Hospital and The Insititue of Cancer Research, London, UK. 3Sarcoma Unit, University of Heidelberg, Mannheim University Medical Center, Mannheim Cancer Center (MCC), Theodor-Kutzer-Ufer 1-3, Mannheim D-68167, Germany.

Publication

Hematol Oncol Clin North Am

Summary

DT is a rare monoclonal fibroblastic neoplasm with an unpredictable biologic behavior. Over the past 15 y, the worldwide approach to desmoid tumor (DT) has shifted dramatically from surgery, when feasible, to frontline active surveillance in the majority of cases, to select patients who truly require treatment. Approximately 50% of cases demonstrate indolent disease, while the remaining patients may require active treatment. Surgery and radiotherapy still have limited indications, but 3 recent randomized trials have provided new insights into medical treatments. This review will highlight the evolution of global concepts and strategies.

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