2nd Department of Internal Medicine - Gastroenterology, Charles University, Faculty of Medicine in Hradec Kralove and University Hospital Hradec Kralove, Czech Republic.
The Fingerland Department of Pathology, Charles University, Faculty of Medicine in Hradec Kralove and University Hospital Hradec Kralove, Czech Republic.
Best Pract Res Clin Gastroenterol. 2021 Mar-Apr;50-51:101728. doi: 10.1016/j.bpg.2021.101728. Epub 2021 Feb 15.
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.
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模式。其特征是胃体 fundic 腺息肉病，并与胃腺癌的高风险相关。与散发性胃癌不同，GAPPS 患者通常不存在幽门螺杆菌。到目前为止，这种相反的发现还没有得到充分阐明。所有存在 fundic 腺息肉病和任何异型增生的 GAPPS 病例均建议预防性全胃切除术。如果组织学检查未发现异型增生，则建议在 30 至 35 岁之间进行预防性胃切除术，或比家族中最年轻的成员发生胃癌的年龄早五年进行预防性胃切除术。GAPPS 的不同表型需要对特定家庭成员采取个体化的方法。
