title: Extra-abdominal desmoid fibromatosis: A review of management, current guidance and unanswered questions.
abstract: Extra abdominal desmoid fibromatosis is a complex condition with many recognised treatments including active observation, hormonal therapy, chemotherapy, radiotherapy and surgical resection. There is large variation in the natural history of individual desmoid tumours, with some cases progressing aggressively and others regressing spontaneously when observed alone. This combined with an absence of accurate clinical predictors of a desmoid tumour's behaviour has led to difficulties in identifying which patients would benefit most from aggressive treatment, and which could be adequately managed with a policy of active observation alone. This review explores the aetiology and common presentation of extra-abdominal desmoid 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 desmoid fibromatosis, and highlight some of the unanswered questions that need to be addressed to optimise the management of this condition.
authors.affiliation: Leicester Royal Infirmary, The University of Leicester NHS Trust, Leicester, LE1 5WW, United Kingdom. Electronic address: neastley@doctors.org.uk.
authors.forename: N
authors.initials: N
authors.lastname: Eastley
authors.affiliation: Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom. Electronic address: Tom.McCulloch@nuh.nhs.uk.
authors.forename: T
authors.initials: T
authors.lastname: McCulloch
authors.affiliation: Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom. Electronic address: Claire.Esler@nuh.nhs.uk.
authors.forename: C
authors.initials: C
authors.lastname: Esler
authors.affiliation: Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom. Electronic address: Ivo.Hennig@nuh.nhs.uk.
authors.forename: I
authors.initials: I
authors.lastname: Hennig
authors.affiliation: Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham, NG5 1PB, United Kingdom. Electronic address: Julia.Fairbairn@nuh.nhs.uk.
authors.forename: J
authors.initials: J
authors.lastname: Fairbairn
authors.affiliation: Istituto Nazionale Tumori, Milan, Italy. Electronic address: Alessandro.Gronchi@istitutotumori.mi.it.
authors.forename: A
authors.initials: A
authors.lastname: Gronchi
authors.affiliation: 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.
authors.forename: R
authors.initials: R
authors.lastname: Ashford
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000032:analysis; D001707:Biopsy, Needle / Q000295:instrumentation / Q000379:methods; D002898:Chromosomes, Human, Pair 8 / Q000235:genetics; D002986:Clinical Trials as Topic; D019468:Disease Management; D005350:Fibroma / Q000175:diagnosis / Q000628:therapy; D018222:Desmoid Tumors / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D006801:Humans; D058990:Molecular Targeted Therapy; D009154:Mutation; D010348:Patient Care Team; D011159:Population Surveillance / Q000379:methods; D017410:Practice Guidelines as Topic; D011379:Prognosis; D012307:Risk Factors; D014314:Trisomy / Q000235:genetics; D051176:beta Catenin / Q000235:genetics
chemical_list: D014408:Biomarkers, Tumor; C495270:CTNNB1 protein, human; D051176:beta Catenin
keywords: Aggressive; Desmoid; Extra-abdominal; Fibromatosis; Management; Review