Best Practice & Research Clinical Gastroenterology ( IF 3.2 ) Pub Date : 2021-02-15 , DOI: 10.1016/j.bpg.2021.101728 Ilja Tacheci , Rudolf Repak , Miroslav Podhola , Lucie Benesova , Jiri Cyrany , Jan Bures , Darina Kohoutova
Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS) is a rare familial gastric cancer syndrome with an autosomal dominant pattern of inheritance. It is characterised by fundic gland polyposis of the gastric body and is associated with a significant risk of gastric adenocarcinoma. Unlike sporadic gastric cancer, Helicobacter pylori is usually absent in patients with GAPPS. This opposite-point finding has so far not been fully clarified. Prophylactic total gastrectomy is indicated in all cases of GAPPS with fundic gland polyposis and the presence of any dysplasia. If no dysplasia is found at histology, prophylactic gastrectomy is suggested at between 30 and 35 years of age, or at five years earlier than the age at which the youngest family member developed gastric cancer. Different phenotypes of GAPPS demand an individual approach to particular family members.
中文翻译:
胃腺癌和胃近端息肉病(GAPPS)–幽门螺杆菌对立点
胃腺癌和胃近端息肉病(GAPPS)是一种罕见的家族性胃癌综合征,具有常染色体显性遗传。其特征在于胃体的胃底腺息肉病,并与胃腺癌的显着风险有关。与散发性胃癌不同,幽门螺杆菌GAPPS患者通常不存在。到目前为止,这种对立的发现还没有完全弄清楚。在所有伴有胃底腺息肉病且存在任何不典型增生的GAPPS病例中,均应进行预防性全胃切除术。如果在组织学上未发现异型增生,建议进行预防性胃切除术的年龄在30至35岁之间,或者比最年轻的家庭成员罹患胃癌的年龄早5岁。GAPPS的不同表型要求针对特定家庭成员的个别方法。