Journal
EUROPEAN JOURNAL OF CANCER
Volume 127, Issue -, Pages 96-107Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.ejca.2019.11.013
Keywords
Desmoid tumour; beta-catenin; CTNNB1; Gardner syndrome; Medical therapy; Radiotherapy; Surgery; Patient advocacy groups; SPAEN
Categories
Funding
- Desmoid Tumor Research Foundation (DTRF)
- sos desmoid Germany
- Sarcoma Patients EuroNet (SPAEN)
Ask authors/readers for more resources
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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available