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

Alman B, Attia S, Baumgarten C, Benson C, Blay JY, Bonvalot S, Breuing J, Cardona K, Casali PG, van Coevorden F, Colombo C, Dei Tos AP, Dileo P, Ferrari A, Fiore M, Frezza AM, Garcia J, Gladdy R, Gounder M, Gronchi A, Haas R, Hackett S, Haller F, Hohenberger P, Husson O, Jones RL, Judson I, Kasper B, Kawai A, Kogosov V, Lazar AJ, Maki R, Mathes T, Messiou C, Navid F, Nishida Y, Palassini E, Penel N, Pollock R, Pieper D, Portnoy M, Raut CP, Roets E, Sandrucci S, Sbaraglia M, Stacchiotti S, Thornton KA, van der Graaf W, van der Zande K, van Houdt WJ, Villalobos V, Wagner AJ, Wardelmann E, Wartenberg M, Watson S, Weiss A, Zafiropoulos N (2020)

Publication Type: Journal article, Review article

Publication year: 2020

Journal

Book Volume: 127

Pages Range: 96-107

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.

Authors with CRIS profile

Involved external institutions

Fondazione IRCCS: Istituto Nazionale dei Tumori Italy (IT) Memorial Sloan Kettering Cancer Center United States (USA) (US) Royal Marsden Hospital / The Royal Marsden NHS Foundation Trust United Kingdom (GB) University College London (UCL) United Kingdom (GB) University of Colorado at Boulder United States (USA) (US) Azienda Ospedaliero Universitaria Città della Salute e della Scienza di Torino Italy (IT) Centre Oscar Lambret (UNICANCER) France (FR) The Institute of Cancer Research (ICR) United Kingdom (GB) Maine Medical Center (MMC) United States (USA) (US) Sarcoma Patients EuroNet e.V./Assoc. (SPAEN) Germany (DE) Netherlands Cancer Institute (NKI) Netherlands (NL) Veterans Affairs Healthcare System Boston and Harvard Medical School United States (USA) (US) Institut Curie France (FR) Dana–Farber Cancer Institute United States (USA) (US) Ospedale di Treviso "Marca Trevigiana" Italy (IT) Université Claude Bernard Lyon 1 (UCB) France (FR) Mayo Clinic Hospital United States (USA) (US) University of Southern California (USC) United States (USA) (US) University of Toronto Canada (CA) Universitätsklinikum Münster Germany (DE) Emory University United States (USA) (US) Ohio State University United States (USA) (US) Desmoid Tumor Research Foundation United States (USA) (US) Sarcoma UK United Kingdom (GB) University of Texas MD Anderson Cancer Center United States (USA) (US) National Cancer Center Hospital Japan (JP) Universitätsklinikum Mannheim Germany (DE) Nagoya University / 名古屋大学 Japan (JP) University of Padua / Universita degli Studi di Padova Italy (IT) Northwell Health United States (USA) (US) European Medicines Agency United Kingdom (GB)

How to cite

APA:

Alman, B., Attia, S., Baumgarten, C., Benson, C., Blay, J.Y., Bonvalot, S.,... Zafiropoulos, N. (2020). The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients. European Journal of Cancer, 127, 96-107. https://doi.org/10.1016/j.ejca.2019.11.013

MLA:

Alman, Ben, et al. "The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients." European Journal of Cancer 127 (2020): 96-107.

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