title: Recurrence and prognostic factors in patients with aggressive fibromatosis. The role of radical surgery and its limitations.
abstract: BACKGROUND
  Surgery is still the standard treatment for aggressive fibromatosis (AF); however, local control remains a significant problem and the impact of R0 surgery on cumulative recurrence (CR) is objective of contradictory reports.
  
  
  METHODS
  This is a single-institution study of 62 consecutive patients affected by extra-abdominal and intra-abdominal AF who received macroscopically radical surgery within a time period of 15 years.
  
  
  RESULTS
  Definitive pathology examination confirmed an R0 situation in 49 patients and an R1 in 13 patients. Five-year CR for patients who underwent R0 vs R1 surgery was 7.1% vs 46.4% (P = 0.04) and for limbs vs other localizations 33.3% vs 9.9% (P = 0.02) respectively. In 17 patients who had intraoperative frozen section (IFS) margin evaluation R0 surgery was more common (17 of 17 vs 32 of 45, P = 0.01) and CR lower (five-year CR 0% vs 19.1%, respectively, P = 0.04). However, in multivariate analysis only limb localization showed a negative impact on CR (HR: 1.708, 95% CI 1.03 to 2.84, P = 0.04).
  
  
  CONCLUSIONS
  IFS evaluation could help the surgeon to achieve R0 surgery in AF. Non-surgical treatment, including watchful follow-up, could be indicated for patients with limb AF localization, because of their high risk of recurrence even after R0 surgery.
authors.affiliation: Division of General and Laparoscopic Surgery, European Institute of Oncology, Milan, Italy. emilio.bertani@ieo.it
authors.forename: Emilio
authors.initials: E
authors.lastname: Bertani
authors.forename: Alessandro
authors.initials: A
authors.lastname: Testori
authors.forename: Antonio
authors.initials: A
authors.lastname: Chiappa
authors.forename: Pasquale
authors.initials: P
authors.lastname: Misitano
authors.forename: Roberto
authors.initials: R
authors.lastname: Biffi
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Viale
authors.forename: Giovanni
authors.initials: G
authors.lastname: Mazzarol
authors.forename: Tommaso
authors.initials: T
authors.lastname: De Pas
authors.forename: Edoardo
authors.initials: E
authors.lastname: Botteri
authors.forename: Gianmarco
authors.initials: G
authors.lastname: Contino
authors.forename: Francesco
authors.initials: F
authors.lastname: Verrecchia
authors.forename: Barbara
authors.initials: B
authors.lastname: Bazolli
authors.forename: Bruno
authors.initials: B
authors.lastname: Andreoni
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D018222:Desmoid Tumors / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality; D009367:Neoplasm Staging; D018365:Neoplasm, Residual / Q000175:diagnosis / Q000209:etiology* / Q000401:mortality; D011379:Prognosis; D012074:Remission Induction; D012307:Risk Factors; D015996:Survival Rate; D055815:Young Adult