Samardali Malik, Samardaly Jehad, Shanti Ibrahim
Internal Medicine, St. Elizabeth Youngstown Hospital - NEOMED (Northeast Ohio Medical University) Program, Youngstown, USA.
Internal Medicine, Jordan University of Science and Technology, Ajloun, JOR.
Cureus. 2025 May 30;17(5):e85072. doi: 10.7759/cureus.85072. eCollection 2025 May.
Hereditary diffuse gastric cancer (HDGC) is a significant genetic predisposition syndrome, primarily caused by germline mutations in the CDH1 gene, which encodes the E-cadherin protein - a critical component for cell adhesion and tissue integrity. Individuals with HDGC have an increased risk of developing diffuse-type gastric cancer, which is characterized by the infiltration of the stomach wall by poorly cohesive, single-cell carcinoma. This aggressive form of gastric cancer is challenging to detect at an early stage and is associated with a poor prognosis. The identification and understanding of the genetic basis of HDGC are crucial for improving early detection, risk assessment, and the development of targeted treatment strategies for this hereditary cancer syndrome. This review aims to provide a comprehensive overview of the genetic and molecular mechanisms underlying HDGC, with a focus on the role of CDH1 mutations. It discusses current diagnostic criteria, genetic testing strategies, and the implications of a positive CDH1 mutation for clinical management - including surveillance, prophylactic gastrectomy, and family counseling. Additionally, this review highlights recent advancements in understanding the impact of CDH1 loss on cellular pathways and the potential for novel therapeutic approaches targeting these molecular changes. By consolidating the existing knowledge and addressing current gaps, this review aims to support clinicians and researchers in enhancing the care and outcomes of individuals and families affected by HDGC.
遗传性弥漫性胃癌(HDGC)是一种重要的遗传易感性综合征,主要由CDH1基因突变引起,该基因编码E-钙黏蛋白——细胞黏附和组织完整性的关键成分。患有HDGC的个体患弥漫型胃癌的风险增加,弥漫型胃癌的特征是胃壁被黏附性差的单细胞癌浸润。这种侵袭性胃癌在早期难以检测,且预后较差。识别和了解HDGC的遗传基础对于改善这种遗传性癌症综合征的早期检测、风险评估以及制定靶向治疗策略至关重要。本综述旨在全面概述HDGC潜在的遗传和分子机制,重点关注CDH1突变的作用。它讨论了当前的诊断标准、基因检测策略以及CDH1突变阳性对临床管理的影响——包括监测、预防性胃切除术和家族咨询。此外,本综述强调了在理解CDH1缺失对细胞通路的影响以及针对这些分子变化的新型治疗方法潜力方面的最新进展。通过整合现有知识并弥补当前差距,本综述旨在支持临床医生和研究人员改善受HDGC影响的个体和家庭的护理及治疗结果。