title: Extra-abdominal primary fibromatosis: Aggressive management could be avoided in a subgroup of patients.
abstract: OBJECTIVE
  To evaluate the impact of surgery as first-line treatment on event-free survival (EFS) of primary aggressive fibromatosis.
  
  
  METHODS
  Treatments were categorized into: surgery with or without radiotherapy and nonsurgical strategies with systemic treatment alone or wait and see policy. Eighty-nine patients had initial resection of their primary tumour followed by postoperative radiotherapy in 13 cases. Twenty-three did not undergo surgery but received systemic treatment or watch and wait policy.
  
  
  RESULTS
  Median follow-up was 76 months. Overall 3 years EFS was 49%. In the univariate analysis, patients with microscopically complete surgery had a similar outcome to patients in the no-surgery group (3 years EFS of 65% and 68%, respectively). Gender, age, tumour size, treatment period and strategy (surgery versus no-surgery) were not statistically significant. Quality of resection according to margins and the tumour site were the only prognostic factors. There was a significant correlation between tumour site and quality of surgery (p=0.0002).
  
  
  CONCLUSIONS
  A subset of patients with extra-abdominal fibromatosis could be managed with a nonaggressive policy, as growth arrest concerned 2/3 of nonoperated patients. When surgery is finally necessary, it should be performed with the aim of achieving negative margins.
authors: Department of Surgery, Institut Gustave Roussy, 39 rue Camille Desmoulins, 94805 Villejuif, France. bonvalot@igr.fr
authors: S
authors: S
authors: Bonvalot
authors: H
authors: H
authors: Eldweny
authors: V
authors: V
authors: Haddad
authors: F
authors: F
authors: Rimareix
authors: G
authors: G
authors: Missenard
authors: O
authors: O
authors: Oberlin
authors: D
authors: D
authors: Vanel
authors: P
authors: P
authors: Terrier
authors: J Y
authors: JY
authors: Blay
authors: A
authors: A
authors: Le Cesne
authors: C
authors: C
authors: Le Péchoux
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D018222:Desmoid Tumors / Q000473:pathology / Q000601:surgery* / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D018714:Radiotherapy, Adjuvant; D012983:Soft Tissue Neoplasms / Q000473:pathology / Q000601:surgery* / Q000628:therapy; D016019:Survival Analysis
chemical_list: D000970:Antineoplastic Agents