The management of desmoid tumours

• Verfasst von: Alman, Ben [VerfasserIn]
• Verfasst von: Hohenberger, Peter [VerfasserIn]
• Titel: The management of desmoid tumours
• Titelzusatz: A joint global consensus-based guideline approach for adult and paediatric patients
• Verf.angabe: the Desmoid Tumor Working Group
• E-Jahr: 2020
• Jahr: 28 January 2020
• Umfang: 12 S.
• Fussnoten: Gesehen am 02.04.2020 ; The Desmoid Tumor Working Group: Alman, Ben; Hohenberger, Peter [und 55 weitere]
• Titel Quelle: Enthalten in: European journal of cancer
• Ort Quelle: Amsterdam [u.a.] : Elsevier, 1992
• Jahr Quelle: 2020
• Band/Heft Quelle: 127(2020), Seite 96-107
• ISSN Quelle: 1879-0852
• DOI: doi:10.1016/j.ejca.2019.11.013
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: aggressive fibromatosis
• Sach-SW: beta-catenin
• Sach-SW: combined surgery
• Sach-SW: ctnnb1
• Sach-SW: Desmoid tumour
• Sach-SW: european organization
• Sach-SW: Gardner syndrome
• Sach-SW: high-dose tamoxifen
• Sach-SW: long-term outcomes
• Sach-SW: Medical therapy
• Sach-SW: Patient advocacy groups
• Sach-SW: phase-ii
• Sach-SW: prognostic-factors
• Sach-SW: radiation-therapy
• Sach-SW: Radiotherapy
• Sach-SW: sarcoma patients euronet
• Sach-SW: spaen
• Sach-SW: Surgery
• Sach-SW: surgical resection
• K10plus-PPN: 1693842122

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 EuropeanOrganisation 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. (C) 2019 The Authors. Published by Elsevier Ltd.