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

Eur J Cancer. 2020 Mar;127:96-107. doi: 10.1016/j.ejca.2019.11.013.

Desmoid Tumor Working Group

Affiliations

1 University of Toronto, Canada.
2 Mayo Clinic, Jacksonville, FL, USA.
3 SOS Desmoid & Sarcoma Patients EuroNet (SPAEN), UK.
4 Royal Marsden Hospital London, London, UK.
5 Centre Léon Bérard & University Cl. Bernard Lyon I, EURACAN, LYRICAN, Lyon, France.
6 Institut Curie, Paris, France.
7 Institute for research in operative medicine, Witten/Herdecke, Germany.
8 Winship Cancer Institute, Emory University, Atlanta, Ga, USA.
9 Fondazione IRCCS Istituto Nazionale dei Tumori & University of Milan, Milan, Italy.
10 The Netherlands Cancer Institute, Amsterdam, the Netherlands.
11 Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
12 University of Padua School of Medicine, Padua, Italy.
13 University College Hospital, UCLH NHS Trust, London, UK.
14 Sarcoma Patients EuroNet (SPAEN), UK.
15 Mount Sinai Hospital and Princess Margaret Cancer Centre, Toronto, Canada.
16 Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, USA.
17 Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. Electronic address: alessandro.gronchi@istitutotumori.mi.it.
18 Sarcoma UK Nurse, London, UK.
19 Friedrich Alexander University Erlangen, Erlangen, Germany.
20 Mannheim University Medical Center, Mannheim, Germany.
21 The Netherlands Cancer Institute, Amsterdam, the Netherlands; The Institute of Cancer Research, London, UK.
22 SOS Desmoid & Sarcoma Patients EuroNet (SPAEN), UK; The Institute of Cancer Research, London, UK.
23 The Institute of Cancer Research, London, UK. Electronic address: bernd.kasper@umm.de.
24 National Cancer Center Hospital, Tokyo, Japan.
25 The Institute of Cancer Research, London, UK.
26 The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
27 Northwell Health Cancer Institute, New York, USA.
28 Children's Hospital Los Angeles, University of Southern California, Los Angeles, USA.
29 Nagoya University Graduate School of Medicine, Nagoya, Japan.
30 Centre Oscar Lambret, Lille, France.
31 The Ohio State University Comprehensive Cancer Center, OH, USA.
32 The Desmoid Tumor Research Foundation (DTRF), Canada.
33 Brigham and Women's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, USA.
34 CDSS University Hospital, Torino, Italy.
35 Azienda ULSS2 Marca Trevigiana, Treviso, Italy.
36 Dana-Farber Cancer Institute and Harvard Medical School, Boston, USA.
37 Division of Medical Oncology, University of Colorado, USA.
38 University Hospital Muenster, Muenster, Germany.
39 Sarcoma PAtients EuroNet (SPAEN), Wölfersheim, Germany.
40 Paediatric Oncology, Maine Medical Center, Portland, USA.
41 European Medicines Agency, London, UK.

PMID: 32004793 DOI: 10.1016/j.ejca.2019.11.013

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.

Keywords

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

Name
Email *

	Sorry, but the email address you supplied was invalid.