Ağaoğlu, Nihat BuğraHatırnaz Ng, ÖzdenZemheri, İtir EbruÜnal, BüşraGerenli, NelginTosun, İlkayYazıcı, HülyaÖzbek, UğurKamihara, JunneRana, Huma Q.2024-10-162024-10-162024Ağaoğlu, N. B., Hatırnaz Ng, Ö., Zemheri, İ. E., Ünal, B., Gerenli, N., Tosun, İ., Yazıcı, H., Özbek, U., Kamihara, J., Rana, H. Q. (2024). Managing CDH1 cancer risks in a child: Complex decision making in a family with hereditary diffuse gastric cancer. American Journal of Medical Genetics Part A. https://doi.org/10.1002/ajmg.a.638971552-48331552-4825https://hdl.handle.net/20.500.13055/821https://doi.org/10.1002/ajmg.a.63897Germline pathogenic variants (PVs) in CDH1 cause hereditary diffuse gastric cancer. The management of CDH1 cases with a positive family history includes total prophylactic gastrectomy or intensive surveillance. In this study, we report a 16-year-old boy with intramucosal gastric signet ring cells in the setting of a germline CDH1 PV and a family history of early-onset gastric cancer. The approach to managing both the proband and their 9-year-old sister, who also had the CDH1 PV, presented a challenge to both clinicians and the family. Herein, we present the complexities of managing gastric cancer risk when a CDH1 PV is identified in childhood in the setting of a family history of early-onset gastric cancer.eninfo:eu-repo/semantics/closedAccessCDH1AdolescentDiffuse Gastric CancerPediatricYoung AdultCase Report10.1002/ajmg.a.63897Q3WOS:0013369809000012-s2.0-85205985224PMID: 39392178Q3