The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients.
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Date
2020-03-01Author
Desmoid Tumor Working Group,
Type
Journal Article
Metadata
Show full item recordAbstract
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.
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Subject
Desmoid Tumor Working Group
Humans
Fibromatosis, Aggressive
Combined Modality Therapy
Consensus
Adult
Child
Disease Management
Practice Guidelines as Topic
Research team
Clinical and Translational Sarcoma
Sarcoma Clinical Trials
Language
eng
Date accepted
2019-11-16
License start date
2020-03
Citation
European journal of cancer (Oxford, England : 1990), 2020, 127 pp. 96 - 107
Publisher
ELSEVIER SCI LTD