Gastrointestinal stromal tumors of the jejunum and ileum: a clinicopathologic, immunohistochemical, and molecular genetic study of 906 cases before imatinib with long-term follow-up.

摘要: Gastrointestinal (GI) stromal tumors (GISTs), the specific KIT- or PDFGRA-signaling driven mesenchymal tumors, are most common of GI tract. This study analyzed 1091 originally classified as smooth muscle small intestine (including jejunum ileum and excluding duodenum), found that 906 (83%) these were GISTs. The GIST patients had 55:45 male-to-female ratio with a median age 59 years (range, 13-94 years). Only 0.6% occurred before 21 13.6% 40 years. varied from 0.3 to cm (median, 7.0 cm) commonly presented bleeding acute abdomen; 18% incidentally detected. Histologically, relatively monotypic spindle cell (86%), epithelioid (5%), mixed patterns (9%). Skeinoid fibers present in 44% cases, their presence was associated favorable course. Most malignant, this morphology sometimes emerged less cellular mitotically active suggesting it represented transformation. KIT immunohistochemically detected 98%, CD34 40%, actin 34%, desmin 0.2%, S-100 protein 14% tested. Outcome strongly dependent on tumor size mitotic activity, an overall 39% tumor-related mortality, twice for gastric 10 >5 mitoses/50 HPF metastasized. In stark contrast corresponding >10 activity 5/50 high metastatic rate (>50%); < = low 24% rate. survival times who died disease decreased by increasing size. exon 11 mutations 90 9 mutation 17 1 case; type not prognostically significant. There no PDGFRA 12 8 mutations. Systematic data prognosis intestinal GISTs various categories can be helpful management surveillance tumors.