title: Clinical characteristics and adequate treatment of familial adenomatous polyposis combined with desmoid tumors.
abstract: OBJECTIVE
  The objective of this study was to examine the clincopathologic characteristics and outcomes of familial adenomatous polyposis (FAP) patients with and without desmoid tumors (DTs), including the risk factors for progression of FAP-related DTs.
  
  
  METHODS
  We reviewed the medical records and database of all patients with FAP who were treated between January 1993 and December 2011.
  
  
  RESULTS
  Of 75 FAP patients, 18 (24%) were FAP with DTs. Seventeen of these had intra-abdominal DTs and one had intra- and extra-abdominal DTs. We divided the patients into two groups according to type of resection; the R0 or R1 resection group, referred to as the curative resection group (eight patients), and the R2 resection/palliative operation/medical treatment group, referred to as the palliative resection group (10 patients). Two patients in the curative resection group and two patients in the palliative group had progressed to tumor growth by the follow-up (p=0.800). In univariate analysis, DT diagnosis before or simultaneously with FAP diagnosis (DTs unrelated to surgical trauma) was a significant risk factor for tumor progression at final follow-up.
  
  
  CONCLUSIONS
  A multidisciplinary approach to DT treatment is needed, including nonsteroidal antiinflammatory drugs, anti-estrogens, cytotoxic agents, and surgery. However, the role of surgery in resectable and complicated tumors may be limited. DT unrelated to surgical trauma has a relatively poor prognosis.
authors.affiliation: Department of Surgery, Institute of Innovative Cancer Research, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
authors.forename: Won Beom
authors.initials: WB
authors.lastname: Jung
authors.affiliation: Department of Surgery, Institute of Innovative Cancer Research, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
authors.forename: Chan Wook
authors.initials: CW
authors.lastname: Kim
authors.affiliation: Department of Surgery, Institute of Innovative Cancer Research, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
authors.forename: Jin Cheon
authors.initials: JC
authors.lastname: Kim
keywords: Aggressive fibromatosis; Familial adenomatous polyposis; Risk factors