Gastrointestinal stromal tumors of the stomach: a clinicopathologic, immunohistochemical, and molecular genetic study of 1765 cases with long-term follow-up.

作者: Markku Miettinen , Leslie H Sobin , Jerzy Lasota

DOI: 10.1097/01.PAS.0000146010.92933.DE

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摘要: Gastrointestinal (GI) stromal tumors (GISTs), the specific KIT- or PDFGRA-signaling driven mesenchymal tumors, are most common of GI tract. In this study, we analyzed 1869 cases originally classified as smooth muscle stomach and found that 1765 (94%) these were GISTs. The GISTs had a slight male predominance (55%) with median age 63 years. Only 2.7% occurred before 21 years 9.1% 40 varied from 0.5 to 44 cm (median, 6.0 cm) commonly presented bleeding; 12% incidentally detected. Several histologic variants recognized among spindle cell (sclerosing, palisaded-vacuolated, hypercellular, sarcomatous) epithelioid dyscohesive, sarcomatous). Outcome was strongly dependent on tumor size mitotic activity. 2% 3% 10 >5 mitoses/50 HPFs metastasized. However, >10 activity 5/50 relatively low metastatic rate (11% 15%). A small number patients survived intra-abdominal metastasis up over 20 Tumor location in fundus gastroesophageal junction, coagulative necrosis, ulceration, mucosal invasion unfavorable factors (P <0.001), whereas antrum favorable <0.001). KIT expression detected 91% cases, CD34 82%, actin 18%, desmin 5%; latter two exon 11 mutations 119 cases; point fared better than those deletions <0.01). PDGFRA 18 (total 86 cases) represented D842V mutation; none prognostically significant. above results may be helpful for setting criteria adjuvant treatment such Gleevec.

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