• Vanessa R Blair, Maybelle McLeod, Fátima Carneiro, Daniel G Coit, Johanna L D'Addario, Jolanda M van Dieren, Kirsty L Harris, Nicoline Hoogerbrugge, Carla Oliveira, Rachel S van der Post, Julie Arnold, Patrick R Benusiglio, Tanya M Bisseling, Alex Boussioutas, Annemieke Cats, Amanda Charlton, Karen E Chelcun Schreiber, Jeremy L Davis, PietroMassimiliano diMDMedical Research Council Cancer Unit, University of Cambridge, Cambridge, UK., Rebecca C Fitzgerald, James M Ford, Kimberley Gamet, Irene Gullo, Richard H Hardwick, David G Huntsman, Pardeep Kaurah, Sonia S Kupfer, Andrew Latchford, Paul F Mansfield, Takeshi Nakajima, Susan Parry, Jeremy Rossaak, Haruhiko Sugimura, Magali Svrcek, Marc Tischkowitz, Toshikazu Ushijima, Hidetaka Yamada, Han-Kwang Yang, Adrian Claydon, Joana Figueiredo, Karyn Paringatai, Raquel Seruca, Nicola Bougen-Zhukov, Tom Brew, Simone Busija, Patricia Carneiro, Lynn DeGregorio, Helen Fisher, Erin Gardner, Tanis D Godwin, Katharine N Holm, Bostjan Humar, Caroline J Lintott, Elizabeth C Monroe, Mark D Muller, Enrique Norero, Yasmin Nouri, Joana Paredes, João M Sanches, Emily Schulpen, Ana S Ribeiro, Andrew Sporle, James Whitworth, Liying Zhang, Anthony E Reeve, and Parry Guilford.
• Department of Surgery, University of Auckland, Auckland, New Zealand; St Marks Breast Centre, Auckland, New Zealand.
• Lancet Oncol. 2020 Aug 1; 21 (8): e386e397e386-e397.

AbstractHereditary diffuse gastric cancer (HDGC) is an autosomal dominant cancer syndrome that is characterised by a high prevalence of diffuse gastric cancer and lobular breast cancer. It is largely caused by inactivating germline mutations in the tumour suppressor gene CDH1, although pathogenic variants in CTNNA1 occur in a minority of families with HDGC. In this Policy Review, we present updated clinical practice guidelines for HDGC from the International Gastric Cancer Linkage Consortium (IGCLC), which recognise the emerging evidence of variability in gastric cancer risk between families with HDGC, the growing capability of endoscopic and histological surveillance in HDGC, and increased experience of managing long-term sequelae of total gastrectomy in young patients. To redress the balance between the accessibility, cost, and acceptance of genetic testing and the increased identification of pathogenic variant carriers, the HDGC genetic testing criteria have been relaxed, mainly through less restrictive age limits. Prophylactic total gastrectomy remains the recommended option for gastric cancer risk management in pathogenic CDH1 variant carriers. However, there is increasing confidence from the IGCLC that endoscopic surveillance in expert centres can be safely offered to patients who wish to postpone surgery, or to those whose risk of developing gastric cancer is not well defined.Copyright © 2020 Elsevier Ltd. All rights reserved.

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