title: Duodenal GIST: a single center experience.
abstract: OBJECTIVE
  The duodenum as primary site for gastrointestinal stromal tumors (GISTs) is rare and mitotic rate, tumor size, type of mutation and number of chromosomal aberrations have prognostic implications.
  
  
  METHODS
  We analyzed the outcome of 13 patients with duodenal GISTs who underwent surgical tumor resection. Either segmental duodenectomy or pylorus-preserving duodenopancreatectomy was performed. The tumors were histopathologically examined and the risk of progression was assessed based on tumor size and mitotic count. Additionally, mutation analysis of the KIT and PDGFRA receptor tyrosine kinase genes and comparative genomic hybridization (CGH) were performed in all cases.
  
  
  RESULTS
  Eight patients underwent segmental duodenectomy and five patients were treated with pylorus-preserving duodenopancreatectomy. None of the five GISTs with low or no risk for malignancy according to the Miettinen classification developed tumor progress. In contrast, five of eight cases (62.5%) with high-risk tumors revealed tumor progress, and four of these patients died (50%). The median overall survival for all patients was 66 months, and the median disease-free survival 41 months. The operative procedure and type of mutation did not correlate with long-term survival. CGH analysis displayed -15q in 12/13 tumors, and -1p in 11/13 cases as characteristic chromosomal aberrations for intestinal origin. Notably, -22q was present in three of four cases with tumor progress.
  
  
  CONCLUSIONS
  Both segmental duodenectomy and pylorus-preserving duodenopancreatectomy are appropriate options to treat duodenal GIST and should be implemented depending on resectability and the patient's performing state. The Miettinen classification and CGH findings correlate with the clinical course.
authors.affiliation: Department of General and Visceral Surgery, University Medical Center Göttingen, Göttingen, Germany.
authors.forename: Alexander
authors.initials: A
authors.lastname: Beham
authors.forename: Inga-Marie
authors.initials: IM
authors.lastname: Schaefer
authors.forename: Silke
authors.initials: S
authors.lastname: Cameron
authors.forename: Katharina
authors.initials: K
authors.lastname: von Hammerstein
authors.forename: Laszlo
authors.initials: L
authors.lastname: Füzesi
authors.forename: Giuliano
authors.initials: G
authors.lastname: Ramadori
authors.forename: Michael B
authors.initials: MB
authors.lastname: Ghadimi
mesh_terms: D000368:Aged; D002869:Chromosome Aberrations; D055028:Comparative Genomic Hybridization; D004252:DNA Mutational Analysis; D013505:Digestive System Surgical Procedures / Q000009:adverse effects; D018572:Disease-Free Survival; D004386:Duodenum / Q000473:pathology* / Q000601:surgery; D005091:Exons / Q000235:genetics; D005260:Female; D005500:Follow-Up Studies; D046152:Gastrointestinal Stromal Tumors / Q000473:pathology* / Q000601:surgery; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000209:etiology; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000235:genetics
chemical_list: D020796:Receptor, Platelet-Derived Growth Factor alpha