title: Extra-abdominal desmoid fibromatosis--a sarcoma unit review of practice, long term recurrence rates and survival.
abstract: BACKGROUND
  Desmoid fibromatosis (DF) carries a significant morbidity and a recognised mortality. Despite this there are currently limited diagnostic or treatment algorithms specific to cases of extra-abdominal DF. Historically surgical excision has formed the cornerstone of treatment. Recently however a paradigm shift has meant many practitioners now adopt a more conservative approach, placing emphasis on active surveillance, function preserving resections, and non-surgical oncologic therapies.
  
  
  METHODS
  We performed an 8-year retrospective review of all cases of extra-abdominal DF managed within our region to assess the consistency of diagnostics, management and long-term outcome.
  
  
  RESULTS
  47 eligible cases were identified. Mean age at diagnosis was 41.3 years (1-81 years). Disease location and speciality of diagnosing practitioners were varied. Management was generally inconsistent. Variation was seen in imaging, biopsy techniques, MDT involvement and management. At a median follow up of 4.9 years our local recurrence rate was 19%.
  
  
  CONCLUSIONS
  The optimal management of DF is unknown. This has led to a lack of formalised guidance for practitioners managing this challenging condition, resulting in inconsistencies and areas for improvement in current management. We propose a diagnostic pathway which may improve consistency of care, reduce potentially unnecessary surgery and the associated morbidity, and significantly increase the rate of complete (R0) surgical resections when surgery is deemed appropriate whilst not significantly worsening oncological outcome. Specifically we propose all cases should be imaged appropriately (usually with MRI), undergo a planned biopsy (by radiologically guided core needle biopsy) and be managed centrally in conjunction with multidisciplinary sarcoma units.
authors.affiliation: Leicester Royal Infirmary, University Hospitals of Leicester NHS Trust, Infirmary Square, Leicester LE1 5WW, United Kingdom. Electronic address: neastley@doctors.org.uk.
authors.forename: N
authors.initials: N
authors.lastname: Eastley
authors.affiliation: Leicester Royal Infirmary, University Hospitals of Leicester NHS Trust, Infirmary Square, Leicester LE1 5WW, United Kingdom. Electronic address: randeep.aujla@hotmail.co.uk.
authors.forename: R
authors.initials: R
authors.lastname: Aujla
authors.affiliation: Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham NG5 1PB, United Kingdom. Electronic address: Robert.Silk@nuh.nhs.uk.
authors.forename: R
authors.initials: R
authors.lastname: Silk
authors.affiliation: Leicester Royal Infirmary, University Hospitals of Leicester NHS Trust, Infirmary Square, Leicester LE1 5WW, United Kingdom. Electronic address: cathy.richards@uhl-tr.nhs.uk.
authors.forename: C J
authors.initials: CJ
authors.lastname: Richards
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 A
authors.initials: TA
authors.lastname: McCulloch
authors.affiliation: Leicester Royal Infirmary, University Hospitals of Leicester NHS Trust, Infirmary Square, Leicester LE1 5WW, United Kingdom; Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham NG5 1PB, United Kingdom. Electronic address: claire.esler@uhl-tr.nhs.uk.
authors.forename: C P
authors.initials: CP
authors.lastname: Esler
authors.affiliation: Leicester Royal Infirmary, University Hospitals of Leicester NHS Trust, Infirmary Square, Leicester LE1 5WW, United Kingdom; Nottingham City Hospital, Nottingham University Hospitals NHS Trust, Hucknall Road, Nottingham NG5 1PB, United Kingdom; Academic Orthopaedics, Trauma & Sports Medicine, University of Nottingham, United Kingdom. Electronic address: robert.ashford@sky.com.
authors.forename: R U
authors.initials: RU
authors.lastname: Ashford
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001132:Arm; D001706:Biopsy; D001943:Breast Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D018222:Desmoid Tumors / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D006258:Head and Neck Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007223:Infant; D007866:Leg; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D012189:Retrospective Studies; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome; D055815:Young Adult
keywords: Aggressive; Desmoid; Diagnosis; Excision; Fibromatosis; Tumour