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

Publication ,  Journal Article
Desmoid Tumor Working Group,
Published in: Eur J Cancer
March 2020

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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Published In

Eur J Cancer

DOI

EISSN

1879-0852

Publication Date

March 2020

Volume

127

Start / End Page

96 / 107

Location

England

Related Subject Headings

  • Practice Guidelines as Topic
  • Oncology & Carcinogenesis
  • Humans
  • Fibromatosis, Aggressive
  • Disease Management
  • Consensus
  • Combined Modality Therapy
  • Child
  • Adult
  • 3211 Oncology and carcinogenesis
 

Citation

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Desmoid Tumor Working Group, . (2020). The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer, 127, 96–107. https://doi.org/10.1016/j.ejca.2019.11.013
Desmoid Tumor Working Group, Stephen D. “The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients.Eur J Cancer 127 (March 2020): 96–107. https://doi.org/10.1016/j.ejca.2019.11.013.
Desmoid Tumor Working Group, Stephen D. “The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients.Eur J Cancer, vol. 127, Mar. 2020, pp. 96–107. Pubmed, doi:10.1016/j.ejca.2019.11.013.
Journal cover image

Published In

Eur J Cancer

DOI

EISSN

1879-0852

Publication Date

March 2020

Volume

127

Start / End Page

96 / 107

Location

England

Related Subject Headings

  • Practice Guidelines as Topic
  • Oncology & Carcinogenesis
  • Humans
  • Fibromatosis, Aggressive
  • Disease Management
  • Consensus
  • Combined Modality Therapy
  • Child
  • Adult
  • 3211 Oncology and carcinogenesis