title: Evaluation of the long term outcome of patients with extremity desmoids.
abstract: BACKGROUND
  The optimum management and long-term outcome for patients with extremity desmoids remains unclear. The aim of this study was to evaluate the long-term outcome of patients with extremity desmoids and to compare patient episodes treated with surgery alone with those treated by radiotherapy either alone or with surgery at any stage of the patient's disease.
  
  
  METHODS
  All patients with extremity desmoids followed at an oncology clinic with an interest in soft tissue tumours and treated between 1959 and 1996 were identified. All were traced back to their primary presentation and first treatment episode.
  
  
  RESULTS
  During the study period 42 patients, 29 females and 13 males were identified. Median follow-up of the patient population was 12.5 years (range 3-30.9). Median age at presentation was 27 years (range 1-69). The lesion was in the upper limb or shoulder in 18, lower limp or pelvis 17 and head and neck 7. Forty patients underwent operation as their primary treatment, 25 of whom developed recurrent tumour. In all there were 41 recurrences following 78 episodes of surgery alone. This compared to 10 recurrences following 16 episodes of radiotherapy or radiotherapy with surgery (p = 0.467). There was no association between primary, first or second recurrence and the development of a further relapse. The median interval to recurrence after surgery alone was 12 months (interquartile range (IQR) 6-13) as opposed to 52 months (IQR 33-62) for patients treated with primary or adjuvant radiotherapy (p= 0.004).
  
  
  CONCLUSIONS
  Results are poor after both radiotherapy and surgery for extremity desmoids and research into other methods of managing this rare condition need to be considered.
authors.affiliation: University Department of Surgery, Western Infirmary, Glasgow G11 6NT, UK.
authors.forename: C
authors.initials: C
authors.lastname: Chew
authors.forename: R
authors.initials: R
authors.lastname: Reid
authors.forename: P J
authors.initials: PJ
authors.lastname: O'Dwyer
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005121:Extremities / Q000601:surgery; D005260:Female; D018222:Desmoid Tumors / Q000532:radiotherapy* / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology; D016896:Treatment Outcome