Gastrointestinale Stromatumoren des Kolorektums und des Ösophagus - Evaluation von Klinik, Pathologie und Outcome
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Background and Objectives: To elucidate diagnostic criteria, clinicopathological features and clinical outcome in patients with esophageal and colorectal gastrointestinal stromal tumors (GIST), representing both rare subforms of GIST. Patients and methods: Esophageal GIST cases from the Ulmer GIST registry consisting of 1077 cases (2006-2012) were pooled with case reports and case series of esophageal GIST extracted from MEDLINE (1993-2013). Data were compared with those from 975 cases with gastric and intestinal GIST from the Ulmer GIST registry. Same method was obtained for colorectal GIST. Analysis of esophageal GIST was main part of this work, analysis of colorectal GIST was just explorative. Results: In comparison to gastric and intestinal GIST, esophageal GIST (n=55) occurred significantly more frequent in men (p=0.028) as well as in patients younger than 60 at diagnosis (p=0.002). Primary tumor sizes were significantly larger (p<0.001), thereby resulting more frequently in a high-risk classification (p<0.001).The 5-year rates of disease-specific survival (DSS), disease-free survival (DFS), and overall survival (OS) were 57 %, 56 % and 54 %, respectively. The prognosis of esophageal GIST was less favorable compared with gastric/intestinal GIST (DSS: p<0.001; DFS: p<0.001; OS p=0.007; univariate Cox model) after a median follow-up time of 28 months (range 1.9 to 202). Mutational analysis for KIT showed more frequently wild-type status in esophageal GIST (p<0.001). In contrast, for most parameters colorectal GIST did not differ significantly from gastric/intestinal GIST. Conclusions: Esophageal GIST differ significantly from gastric/intestinal GIST in respect to clinicopathological features and clinical outcome. To optimize treatment options further prospective data on patients with esophageal GIST are urgently warranted.
Erstellung / Fertigstellung
Normierte SchlagwörterDNA mutational analysis [MeSH]
Gastrointestinal stromal tumor [MeSH]
Treatment outcome [MeSH]