title: Desmoid tumor: analysis of prognostic factors and outcomes in a surgical series.
abstract: BACKGROUND
  Desmoid tumors are rare and exhibit a highly unpredictable natural history. We sought to analyze prognostic factors associated with recurrence in a large single-institution study of patients with desmoid tumors.
  
  
  METHODS
  We performed a retrospective review of 177 patients with desmoid tumor who underwent macroscopically complete surgical resection, with or without the addition of radiotherapy (RT) or systemic therapy, from 1970 to 2009. We examined patterns of presentation, all known risk factors for recurrence, and their association with recurrence-free survival (RFS).
  
  
  RESULTS
  Twenty-two patients (12 %) had intra-abdominal desmoid tumors, and 155 (88 %) had extra-abdominal tumors. Patterns of presentation included primary (n = 133, 75 %) and locally recurrent (n = 44, 25 %) disease. Treatment was surgery alone in 125 patients (71 %), surgery and RT in 36 (20 %), and surgery and systemic therapy with or without RT in 20 (11 %). Median follow-up was 40 months. Overall, the local relapse rate was 29 %, and 10-year RFS was 60 %. R0 resection status was the only predictor of freedom from local recurrence on multivariate analysis (odds ratio 0.32; 95 % confidence interval 0.15-0.66; P = 0.002). The selective use of adjuvant RT appeared to improve local control in patients with positive margins.
  
  
  CONCLUSIONS
  For patients with desmoid tumors undergoing surgery, wide excision with negative margins should be the goal, but not at the expense of function, as fewer than half of patients with positive margins will experience recurrence.
authors.affiliation: Division of Surgical Oncology, Massachusetts General Hospital, Boston, MA, USA. jmullen@partners.org
authors.forename: John T
authors.initials: JT
authors.lastname: Mullen
authors.forename: Thomas F
authors.initials: TF
authors.lastname: Delaney
authors.forename: Wendy K
authors.initials: WK
authors.lastname: Kobayashi
authors.forename: Jackie
authors.initials: J
authors.lastname: Szymonifka
authors.forename: Beow Y
authors.initials: BY
authors.lastname: Yeap
authors.forename: Yen-Lin
authors.initials: YL
authors.lastname: Chen
authors.forename: Andrew E
authors.initials: AE
authors.lastname: Rosenberg
authors.forename: David C
authors.initials: DC
authors.lastname: Harmon
authors.forename: Edwin
authors.initials: E
authors.lastname: Choy
authors.forename: Sam S
authors.initials: SS
authors.lastname: Yoon
authors.forename: Kevin A
authors.initials: KA
authors.lastname: Raskin
authors.forename: G
authors.initials: G
authors.lastname: Petur Nielsen
authors.forename: Francis J
authors.initials: FJ
authors.lastname: Hornicek
mesh_terms: D000328:Adult; D003131:Combined Modality Therapy; D005260:Female; D018221:Fibromatosis, Abdominal / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D018222:Desmoid Tumors / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000401:mortality* / Q000473:pathology / Q000628:therapy; D011183:Postoperative Complications; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D015996:Survival Rate