β-Catenin activation in fundic gland polyps, gastric cancer and colonic polyps in families afflicted by 'gastric adenocarcinoma and proximal polyposis of the stomach' (GAPPS).

β-Catenin activation in fundic gland polyps, gastric cancer and colonic polyps in families afflicted by 'gastric adenocarcinoma and proximal polyposis of the stomach' (GAPPS).
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DOI:
10.1136/jclinpath-2016-203746
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发表时间:
2016-09
影响因子:
3.4
通讯作者:
Rudloff U
Rudloff U
中科院分区:
医学3区
文献类型:
--
作者:
McDuffie LA;Sabesan A;Allgäeuer M;Xin L;Koh C;Heller T;Davis JL;Raffeld M;Miettienen M;Quezado M;Rudloff U

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目的:探讨胃腺癌和胃近端息肉病(GAPPS)胃肠息肉综合征(GAPPS)中结肠受累的可能性。两个GAPPS家系的前瞻性临床病理评价及免疫组织化学方法检测胃镜和手术标本中β-连环蛋白、P53和Ki67的表达。具有GAPPS表型的患者比同一家族中的高危患者更容易受到结肠息肉的影响(p<0.01)。结肠息肉与胃底腺息肉和胃癌具有相同的免疫组织化学特征,包括核β-连环蛋白、Ki67和P53的高表达。胃和结肠病变都含有激活的β-连环蛋白信号的体细胞变体。胃底腺和结肠息肉中表达标志物的相似性,以及受GAPPS表型影响的家族成员结肠腺瘤比高危家族成员更多,支持这种罕见癌症综合征的轻度结肠受累。结肠镜检查可能是有必要的。#09-C-0079;结果
To evaluate possible colon involvement in the ‘gastric adenocarcinoma and proximal polyposis of the stomach’ (GAPPS) gastrointestinal polyposis syndrome. Prospective clinicopathological evaluation of two GAPPS families and expression of nuclear β-catenin, p53 and Ki67 measured by immunohistochemistry on endoscopic and surgical specimens from patients with GAPPS. Patients with the GAPPS phenotype were more frequently affected by colonic polyps than patients at risk within the same families (p<0.01). Colonic polyps shared immunohistochemical features of fundic gland polyps and gastric cancers including increased expression of nuclear β-catenin, Ki67 and p53. Both gastric and colonic lesions harboured activating somatic variants of β-catenin signalling. Similarities in expression markers in fundic gland and colonic polyps, together with an enrichment of colonic adenomas in family members affected by GAPPS phenotype compared with family members at risk, support mild colonic involvement of this rare cancer syndrome. Colonoscopic screening might be warranted. #09-C-0079; Results.