The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients

Ben Alman, Steven Attia, Christina Baumgarten, Charlotte Benson, Jean Yves Blay, Sylvie Bonvalot, Jessica Breuing, Ken Cardona, Paolo G. Casali, Frits van Coevorden, Chiara Colombo, Angelo P. Dei Tos, Palma Dileo, Andrea Ferrari, Marco Fiore, Anna M. Frezza, Jesica Garcia, Rebecca Gladdy, Mrinal Gounder, Alessandro GronchiRick Haas, Sam Hackett, Florian Haller, Peter Hohenberger, Olga Husson, Robin L. Jones, Ian Judson, Bernd Kasper, Akira Kawai, Vlada Kogosov, Alex J. Lazar, Robert Maki, Tim Mathes, Christina Messiou, Fariba Navid, Yoshihiro Nishida, Elena Palassini, Nicolas Penel, Robert Pollock, Dawid Pieper, Marlene Portnoy, Chandrajit P. Raut, Evelyne Roets, Sergio Sandrucci, Marta Sbaraglia, Silvia Stacchiotti, Katherine A. Thornton, Winette van der Graaf, Kim van der Zande, Winan J. van Houdt, Victor Villalobos, Andrew J. Wagner, Eva Wardelmann, Markus Wartenberg, Sarah Watson, Aaron Weiss, Nikolaos Zafiropoulos

Research output: Contribution to journalReview articlepeer-review

22 Scopus citations

Abstract

Desmoid tumor (DT; other synonymously used terms: Desmoid-type fibromatosis, aggressive fibromatosis) is a rare and locally aggressive monoclonal, fibroblastic proliferation characterised by a variable and often unpredictable clinical course. Previously surgery was the standard primary treatment modality; however, in recent years a paradigm shift towards a more conservative management has been introduced and an effort to harmonise the strategy amongst clinicians has been made. We present herein an evidence-based, joint global consensus guideline approach to the management of this disease focussing on: molecular genetics, indications for an active treatment, and available systemic therapeutic options. This paper follows a one-day consensus meeting held in Milan, Italy, in June 2018 under the auspices of the European Reference Network for rare solid adult cancers, EURACAN, the European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG) as well as Sarcoma Patients EuroNet (SPAEN) and The Desmoid tumour Research Foundation (DTRF). The meeting brought together over 50 adult and pediatric sarcoma experts from different disciplines, patients and patient advocates from Europe, North America and Japan.

Original languageEnglish (US)
Pages (from-to)96-107
Number of pages12
JournalEuropean Journal of Cancer
Volume127
DOIs
StatePublished - Mar 2020

Keywords

  • CTNNB1
  • Desmoid tumour
  • Gardner syndrome
  • Medical therapy
  • Patient advocacy groups
  • Radiotherapy
  • SPAEN
  • Surgery
  • Treatment algorithm
  • β-catenin

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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