Abstract
PurposeThe 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.MethodsWe 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.ResultsEight 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.ConclusionsBoth 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.
Highlights
Gastrointestinal stromal tumors (GISTs) are supposed to arise from the interstitial cells of Cajal or their precursors, located throughout the muscular wall of the gastrointestinal tract
The Miettinen classification and comparative genomic hybridization (CGH) findings correlate with the clinical course
The surgical procedure, including segmental duodenectomy or pylorus-preserving partial duodenopancreatectomy according to Traverso-Longmire was registered
Summary
Gastrointestinal stromal tumors (GISTs) are supposed to arise from the interstitial cells of Cajal or their precursors, located throughout the muscular wall of the gastrointestinal tract. They occur at an incidence of 10–20/million per year and at a median age of 55–60 years [1,2,3,4]. But 5% occur in the context of a familial syndrome (i.e., neurofibromatosis type 1, Carney triad) [2]. They usually present with abdominal pain to due obstruction, anemia, or
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