Current Management of Desmoid Tumors

作者：Bernd Kasper, Elizabeth Healey Baldini, Sylvie Bonvalot, Dario Callegaro, Kenneth Cardona, Chiara Colombo, Nadège Corradini, Aimee Marie Crago, Angelo Paolo Dei Tos, Palma Dileo, Eldad Elnekave, Joseph Patrick Erinjeri, Fariba Navid, Jeffrey M. Farma, Andrea C. Ferrari, Marco Fiore, Rebecca Anne Gladdy, Mrinal M. Gounder, Rick L. Haas, Olga Husson, Jean‐Emmanuel Kurtz, Alex J. Lazar, Daniel Orbach, Nicolas Penel, R. Ratan, Chandrajit P. Raut, Christina Lynn Roland, Anne‐Rose W. Schut, Monika Sparber‐Sauer, D. Strauß, Winette T.A. van der Graaf, Marco Vitellaro, Aaron R. Weiss, Alessandro Gronchi, Desmoid Tumor Working Group, Benjamin Aaron Alman, Steven Attia, Jyoti Bajpai, Giacomo Giulio Baldi, Livia Prudente Barbieri, Christina Baumgarten, Charlotte Benson, Jean‐Yves Blay, Kjetil Boye, Bernadette Brennan, Andre Domenico Campanacci, Paolo Giovanni Casali, Michela Casanova, Tom Wie-Wu Chen, Lorenzo D’Ambrosio, Chaves Maria Carolina De Menezes, Armelle Dufresne, Hans Roland Dürr, Anna Maria Frezza, David E. Gyorki, Sam Hackett, Florian Haller, Audrey Hélaine, Peter Hohenberger, Toni Ibrahim, Shintaro Iwata, Robin L. Jones, Akira Kawai, Mohamed Kelany, David G. Kirsch, Andreas Leithner, Andrea J. MacNeill, Christina Messiou, Robert G. Maki, Carlo Morosi, Andrea Napolitano, Yoshihiro Nishida, Elena Palassini, SHREYASKUMAR R. PATEL, Giulia Personeni, Raphael E. Pollock, Emanuele Rausa, Evelyne Roets, Enrica Rossi, Piotr Lukasz Rutkowski, Daniela Salvatore, Claudia Sangalli, Marta Sbaraglia, Philip Michael Smith, Silvia Stacchiotti, William D. Tap, Gabriel Tinoco, Dimitri Tzanis, Michiel A. J. van de Sande, Kim van der Zande, Winan J. van Houdt, Cornelis Kees Verhoef, Andrew J. Wagner, Eva Wardelmann, Jeanne Whiting · 发表于：JAMA Oncology · 年份：2024 · DOI：10.1001/jamaoncol.2024.1805 · 被引用次数：109 · 研究领域：Soft tissue tumor case studies、Soft tissue tumors and treatment、Sarcoma Diagnosis and Treatment

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 retrieve...