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Gastric cancer is the third most common cancer worldwide. The majority of gastric cancers are sporadic but familial clustering is seen in more than 10% of cases. This manuscript is divided into two parts. The first part is dedicated to the non-syndromic hereditary gastric cancer, particularly the hereditary diffuse gastric cancer (HDGC) and other gastric polyposes including the recently described GAPPS (Gastric adenocarcinoma and proximal polyposis of the stomach). The second part concerns the syndromic gastric cancer, namely the HNPCC syndrome (Hereditary Non Polyposis Colorectal Cancer) occurring as part of a genetic predisposition syndrome to cancer. Recent advances in oncogenetics and next generation sequencing technology have enabled the identification of new entities. This enhancement in knowledge regarding inherited syndromes predisposing to gastric cancer has consequently improved the management of patients and their families. In this context, pathologists play a major role in identifying particular morphologic entities prompting genetic investigation. The aim of this manuscript is to provide an update on the current knowledge about hereditary gastric cancer., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Patients with hereditary predisposition to digestive cancer are at high risk of neoplasia and management in expert centers is recommended. The PRED-IdF network was thus created in 2009, with the support of the French National Cancer Institute (INCa), covering Paris and its suburbs, including five teaching hospitals and two oncology-dedicated institutes. The aim of this network is to offer optimized cancer screening programs based on expert recommendations to patients with hereditary predisposition. Any patient with suspicion of hereditary colorectal syndrome can be referred to the PRED-IdF network. The missions of this network include the establishment of a personalized screening program (PSP), coordination of PSP, expertise/recourse for difficult cases and research. Since 2009, 3384 patients have been included. We genetically identified 1925 patients with Lynch syndrome and 539 with familial adenomatous polyposis (FAP) (including both APC and MUTYH mutations), representing 72.8% of the PRED-IdF cohort. The PRED-IdF is also an important promotor of research in the field. We recently demonstrated the beneficial impact of the network in terms of colorectal cancer occurrence in patients with Lynch syndrome. Moreover, the PRED-IdF is involved in many studies ranging from basic science collaborations to randomized controlled trials. The long-term objective is to offer to all patients a personalized medical approach., (Copyright © 2020. Published by Elsevier Masson SAS.)