The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients.
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ICR Authors
Authors
Desmoid Tumor Working Group,
Document Type
Journal Article
Date
2020-03-01
Date Accepted
2019-11-16
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.
Citation
European journal of cancer (Oxford, England : 1990), 2020, 127 pp. 96 - 107
Source Title
Publisher
ELSEVIER SCI LTD
ISSN
0959-8049
eISSN
1879-0852
Collections
Research Team
Clinical and Translational Sarcoma
Sarcoma Clinical Trials
Sarcoma Clinical Trials