title: Management and recurrence patterns of desmoids tumors: a multi-institutional analysis of 211 patients.
abstract: BACKGROUND
  Desmoid tumors are rare soft-tissue neoplasms with limited data on their management. We sought to determine the rates of recurrence following surgery for desmoid tumors and identify factors predictive of disease-free survival.
  
  
  METHODS
  Between January 1983 and December 2011, 211 patients with desmoid tumors were identified from three major surgical centers. Clinicopathologic and treatment characteristics were analyzed to identify predictors of recurrence.
  
  
  RESULTS
  Median age was 36 years; patients were predominantly female (68 %). Desmoid tumors most commonly arose in extremities (32 %), abdominal cavity (23 %) or wall (21 %), and thorax (15 %); median size was 7.5 cm. Most patients had an R0 surgical margin (60 %). The 1- and 5-year recurrence-free survival was 81.3 and 52.8 %, respectively. Factors associated with worse recurrence-free survival were: younger age (for each 5-year increase in age, hazard ratio [HR] = 0.90, 95 % confidence interval [95 % CI] 0.82-0.98) and extra-abdominal tumor location (abdominal wall referent: extra-abdominal site, HR = 3.28, 95 % CI, 1.46-7.36) (both P < 0.05).
  
  
  CONCLUSIONS
  Recurrence remains a problem following resection of desmoid tumors with as many as 50 % of patients experiencing a recurrence within 5 years. Factors associated with recurrence included age, tumor location, and margin status. While surgical resection remains central to the management of patients with desmoid tumors, the high rate of recurrence highlights the need for more effective adjuvant therapies.
authors.affiliation: Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
authors.forename: Peter D
authors.initials: PD
authors.lastname: Peng
authors.forename: Omar
authors.initials: O
authors.lastname: Hyder
authors.forename: Michael N
authors.initials: MN
authors.lastname: Mavros
authors.forename: Ryan
authors.initials: R
authors.lastname: Turley
authors.forename: Ryan
authors.initials: R
authors.lastname: Groeschl
authors.forename: Amin
authors.initials: A
authors.lastname: Firoozmand
authors.forename: Michael
authors.initials: M
authors.lastname: Lidsky
authors.forename: Joseph M
authors.initials: JM
authors.lastname: Herman
authors.forename: Michael
authors.initials: M
authors.lastname: Choti
authors.forename: Nita
authors.initials: N
authors.lastname: Ahuja
authors.forename: Robert
authors.initials: R
authors.lastname: Anders
authors.forename: Daniel G
authors.initials: DG
authors.lastname: Blazer
authors.forename: T Clark
authors.initials: TC
authors.lastname: Gamblin
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Pawlik
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D018221:Fibromatosis, Abdominal / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D018222:Desmoid Tumors / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D011183:Postoperative Complications; D011379:Prognosis; D015996:Survival Rate; D055815:Young Adult