title: A nonrandom association of gastrointestinal stromal tumor (GIST) and desmoid tumor (deep fibromatosis): case series of 28 patients.
abstract: BACKGROUND
  Gastrointestinal stromal tumors (GISTs) and desmoid tumors (DTs) are two rare mesenchymal tumor. Anecdotal reports of individuals with both diseases led us to make the hypothesis that the association is a nonrandom event as the probability would be extremely low to observe such cases if they were independent events.
  
  
  METHODS
  We evaluated the existence of patients with GIST and DT in a large multicenter cohort at 10 institutions in the United States, Australia and Europe. Data on gender, age at diagnosis, KIT, PDGFRA, CTNNB1 mutation status and follow-up time after diagnosis were collected.
  
  
  RESULTS
  We identified 28 patients diagnosed with both tumors. DT was diagnosed after GIST in 75% of patients and concomitantly in 21%. In only one case (4%), GIST was diagnosed after DT. KIT or PDGFRA mutations were detected in 12 of 14 GIST, 9 in KIT exon 11, 2 in KIT exon 9 and 1 in PDGFRA.
  
  
  CONCLUSIONS
  A statistical analysis of these 28 cases suggests a nonrandom association between GIST and DT. Further studies may be able to elucidate the underlying biology responsible for this association.
authors: Department of Sarcoma Medical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston.
authors: A G
authors: AG
authors: Dumont
authors: Department of Medical Oncology, Fox Chase Cancer Center, Philadelphia.
authors: L
authors: L
authors: Rink
authors: Department of Medical Oncology, Fox Chase Cancer Center, Philadelphia; Department of Pathology and Laboratory Medicine, University of Kansas Medical Center.
authors: A K
authors: AK
authors: Godwin
authors: Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, USA.
authors: M
authors: M
authors: Miettinen
authors: Department of Oncology, Helsinki University Central Hospital, Helsinki, Finland.
authors: H
authors: H
authors: Joensuu
authors: Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center, Tampa, USA.
authors: J R
authors: JR
authors: Strosberg
authors: Department of Surgery, National Tumor Institute, Milan, Italy.
authors: A
authors: A
authors: Gronchi
authors: Department of Pathology and Knight Cancer Institute, Oregon Health & Science University, Portland, USA.
authors: C L
authors: CL
authors: Corless
authors: Department of Medical Oncology, Prince of Wales Hospital, Sydney, Australia.
authors: D
authors: D
authors: Goldstein
authors: Department of Molecular Genetics, Lerner Research Institute; Department of Anatomic Pathology, Taussig Cancer Center, Cleveland Clinic, Cleveland.
authors: B P
authors: BP
authors: Rubin
authors: Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York.
authors: R G
authors: RG
authors: Maki
authors: Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston.
authors: A J
authors: AJ
authors: Lazar
authors: Department of Cancer Biology, The University of Texas M. D. Anderson Cancer Center, Houston, USA.
authors: D
authors: D
authors: Lev
authors: Department of Sarcoma Medical Oncology, The University of Texas M. D. Anderson Cancer Center, Houston. Electronic address: jonctrent@gmail.com.
authors: J C
authors: JC
authors: Trent
authors: Department of Medical Oncology, Fox Chase Cancer Center, Philadelphia.
authors: M
authors: M
authors: von Mehren
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001315:Australia / Q000453:epidemiology; D015331:Cohort Studies; D005060:Europe / Q000453:epidemiology; D005260:Female; D018222:Desmoid Tumors / Q000150:complications* / Q000453:epidemiology*; D005500:Follow-Up Studies; D046152:Gastrointestinal Stromal Tumors / Q000150:complications* / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014481:United States / Q000453:epidemiology