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The Desmoid Tumor Research Foundation

February 2016

Extra-abdominal desmoid fibromatosis: A review of management, current guidance and unanswered questions.

Author Information

A Gronchi,C Esler,I Hennig,J Fairbairn,N Eastley,R Ashford,T McCulloch

Istituto Nazionale Tumori, Milan, Italy. Electronic address: Alessandro.Gronchi@istitutotumori.mi.it.,Leicester Royal Infirmary, The University of Leicester NHS Trust, Leicester, LE1 5WW, United Kingdom. Electronic address: neastley@doctors.org.uk.,Leicester Royal Infirmary, The University of Leicester NHS Trust, Leicester, LE1 5WW, United Kingdom; Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom. Electronic address: Robert.Ashford@uhl-tr.nhs.uk.,Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom. Electronic address: Claire.Esler@nuh.nhs.uk.,Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom. Electronic address: Ivo.Hennig@nuh.nhs.uk.,Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom. Electronic address: Julia.Fairbairn@nuh.nhs.uk.,Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom. Electronic address: Tom.McCulloch@nuh.nhs.uk.

Publication

Summary

This review explores the aetiology and common presentation of extra-abdominaldesmoid fibromatosis including the condition's histopathological, clinical and radiological characteristics. The current evidence for potential predictors of desmoid tumour behaviour is also reviewed, along with the indications and evidence for the multitude of treatments available. We also summarise the published guidelines that are currently available for oncologists and surgeons managing extra-abdominal desmoidfibromatosis, and highlight some of the unanswered questions that need to be addressed to optimise the management of this condition.

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