Current Management of Desmoid Tumors (2024)

• ¹Sarcoma Unit, Mannheim Cancer Center (MCC), Mannheim University Medical Center, University of Heidelberg, Mannheim, Germany

• ²Sarcoma Center, Dana-Farber Cancer Institute and Brigham and Women’s Hospital, Boston, Massachusetts

• ³Department of Surgery, Institut Curie, Comprehensive Cancer Center, Paris, France

• ⁴Department of Surgery, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Istituto Nazionale dei Tumori, Milan, Italy

• ⁵Division of Surgical Oncology, Department of Surgery, Emory University School of Medicine and Winship Cancer Institute, Atlanta, Georgia

• ⁶Department of Pediatric Oncology, Institut d’Hématologie et d’Oncologie Pédiatrique, Centre Léon Bérard, Lyon, France

• ⁷Department of Surgery, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York

• ⁸Surgical Pathology & Cytopathology Unit, Department of Medicine (DIMED), University of Padua, Padua, Italy

• ⁹Department of Oncology, University College London Hospitals National Health Service (NHS) Foundation Trust, London, United Kingdom

• ¹⁰Unit of Interventional Radiology, Shaare Tzedek Medical Center, Jerusalem, Israel

• ¹¹Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, New York

• ¹²Department of Pediatrics, Division of Hematology-Oncology, Cancer and Blood Disease Institute, Children’s Hospital Los Angeles, Los Angeles, California

• ¹³Department of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania

• ¹⁴Deparment of Pediatrics, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

• ¹⁵Division of Surgical Oncology, Department of Surgery, University of Toronto, Ontario, Canada

• ¹⁶Department of Surgery, Mount Sinai Hospital, Sinai Health Systems, Toronto, Ontario, Canada

• ¹⁷Princess Margaret Cancer Centre, University Health Network, Toronto, Ontario, Canada

• ¹⁸Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York

• ¹⁹Department of Radiotherapy, the Netherlands Cancer Institute, Amsterdam, the Netherlands

• ²⁰Department of Radiotherapy, Leiden University Medical Center, Leiden, the Netherlands

• ²¹Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, the Netherlands

• ²²Department of Medical and Surgical Oncology & Hematology, Institut de Cancérologie Strasbourg Europe (ICANS), Strasbourg, France

• ²³Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston

• ²⁴SIREDO (Care, Innovation & Research in Childhood, Adolescent & Young-Adult Oncology) Oncology Center, Institut Curie, Paris Sciences et Lettres University, Paris, France

• ²⁵Department of Medical Oncology, Centre Oscar Lambret, Lille, France

• ²⁶Department of Sarcoma Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston

• ²⁷Department of Surgery, Brigham and Women’s Hospital, Boston, Massachusetts

• ²⁸Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston

• ²⁹Department of Surgical Oncology, Erasmus MC Cancer Institute, Rotterdam, the Netherlands

• ³⁰Stuttgart Cancer Center, Zentrum für Kinder- und Jugend Frauenmedizin, Pädiatrie 5, Klinikum Stuttgart, Stuttgart, Germany

• ³¹Sarcoma and Melanoma Unit, Department of Academic Surgery, The Royal Marsden NHS Foundation Trust, London, United Kingdom

• ³²Division of Hematology-Oncology, Department of Pediatrics, Maine Medical Center, Portland

Importance Desmoid tumor (DT) is a rare and locally aggressive monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Previously, surgery was the standard primary treatment modality; however, within the past decade, a paradigm shift toward less-invasive management has been introduced and an effort to harmonize the strategy among clinicians has been made. To update the 2020 global evidence-based consensus guideline on the management of patients with DT, the Desmoid Tumor Working Group convened a 1-day consensus meeting in Milan, Italy, on June 30, 2023, under the auspices of the European Reference Network on Rare Adult Solid Cancers and Sarcoma Patient Advocacy Global Network, the Desmoid Foundation Italy, and the Desmoid Tumor Research Foundation. The meeting brought together over 90 adult and pediatric sarcoma experts from different disciplines as well as patients and patient advocates from around the world.

Observations The 2023 update of the global evidence-based consensus guideline focused on the positioning of local therapies alongside surgery and radiotherapy in the treatment algorithm as well as the positioning of the newest class of medical agents, such as γ-secretase inhibitors. Literature searches of MEDLINE and Embase databases were performed for English-language randomized clinical trials (RCTs) of systemic therapies to obtain data to support the consensus recommendations. Of the 18 full-text articles retrieved, only 4 articles met the inclusion criteria. The 2023 consensus guideline is informed by a number of new aspects, including data for local ablative therapies such as cryotherapy; other indications for surgery; and the γ-secretase inhibitor nirogacestat, the first representative of the newest class of medical agents and first approved drug for DT. Management of DT is complex and should be carried out exclusively in designated DT referral centers equipped with a multidisciplinary tumor board. Selection of the appropriate strategy should consider DT-related symptoms, associated risks, tumor location, disease morbidities, available treatment options, and preferences of individual patients.

Conclusions and Relevance The therapeutic armamentarium of DT therapy is continually expanding. It is imperative to carefully select the management strategy for each patient with DT to optimize tumor control and enhance quality of life.

Pediatric Cancer Surgical Oncology Health Care Quality Surgery Oncology Guidelines Radiation Oncology Sarcomas Soft-Tissue Tumors Pediatrics Substance Use and Addiction Medicine Alcohol

Citation

Kasper B, Baldini EH, Bonvalot S, et al. Current Management of Desmoid Tumors: A Review. JAMA Oncol. Published online June 20, 2024. doi:10.1001/jamaoncol.2024.1805

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