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Hereditary diffuse gastric cancer: updated clinical practice guidelines.

ABSTRACT: Hereditary 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.

SUBMITTER: Blair VR

PROVIDER: S-EPMC7116190 | biostudies-literature | 2020 Aug

REPOSITORIES: biostudies-literature

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Hereditary diffuse gastric cancer: updated clinical practice guidelines.

Blair Vanessa R VR McLeod Maybelle M Carneiro Fátima F Coit Daniel G DG D'Addario Johanna L JL van Dieren Jolanda M JM Harris Kirsty L KL Hoogerbrugge Nicoline N Oliveira Carla C van der Post Rachel S RS Arnold Julie J Benusiglio Patrick R PR Bisseling Tanya M TM Boussioutas Alex A Cats Annemieke A Charlton Amanda A Schreiber Karen E Chelcun KEC Davis Jeremy L JL Pietro Massimiliano di MD Fitzgerald Rebecca C RC Ford James M JM Gamet Kimberley K Gullo Irene I Hardwick Richard H RH Huntsman David G DG Kaurah Pardeep P Kupfer Sonia S SS Latchford Andrew A Mansfield Paul F PF Nakajima Takeshi T Parry Susan S Rossaak Jeremy J Sugimura Haruhiko H Svrcek Magali M Tischkowitz Marc M Ushijima Toshikazu T Yamada Hidetaka H Yang Han-Kwang HK Claydon Adrian A Figueiredo Joana J Paringatai Karyn K Seruca Raquel R Bougen-Zhukov Nicola N Brew Tom T Busija Simone S Carneiro Patricia P DeGregorio Lynn L Fisher Helen H Gardner Erin E Godwin Tanis D TD Holm Katharine N KN Humar Bostjan B Lintott Caroline J CJ Monroe Elizabeth C EC Muller Mark D MD Norero Enrique E Nouri Yasmin Y Paredes Joana J Sanches João M JM Schulpen Emily E Ribeiro Ana S AS Sporle Andrew A Whitworth James J Zhang Liying L Reeve Anthony E AE Guilford Parry P

The Lancet. Oncology 20200801 8

Hereditary 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), wh ...[more]

PMID: 32758476

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Project description:OBJECTIVE:Guidelines on safe use of iodinated contrast material recommend intravenous prophylactic hydration to prevent post-contrast adverse (renal) effects. Recently, guidelines have been updated and standard prophylaxis is no longer recommended for the majority of patients. The current study aims to evaluate the consequences for clinical practice of the updated guidelines in terms of complications, hospitalisations, and costs. METHODS:The Contrast-Induced Nephropathy After Reduction of the prophylaxis Threshold (CINART) project is a retrospective observational study. All elective procedures with intravascular iodinated contrast administration at Maastricht University Medical Centre (UMC+) in patients aged >?18 years, formerly eligible for prophylaxis (eGFR 30-44 ml/min/1.73 m2 or eGFR 45-59 ml/min/1.73 m2 in combination with diabetes or >?1 predefined risk factor), and currently eligible for prophylaxis (eGFR <?30 ml/min/1.73 m2) were included. Data were used to calculate relative reductions in complications, hospitalisations, and costs associated with standard prophylactic intravenous hydration. CINART is registered with Clinicaltrials.gov: NCT03227835. RESULTS:Between July 1, 2017, and July 1, 2018, 1992 elective procedures with intravascular iodinated contrast in patients formerly and currently eligible for prophylaxis were identified: 1808 in patients formerly eligible for prophylaxis and 184 in patients currently eligible for prophylaxis. At Maastricht UMC+, guideline updates led to large relative reductions in numbers of complications of prophylaxis (e.g. symptomatic heart failure; -?89%), extra hospitalisations (-?93%), and costs (-?91%). CONCLUSION:Guideline updates have had a demonstrable impact on daily clinical practice benefiting patient, hospital, and health care budgets. Clinical practice varies between institutions and countries; therefore, a local estimation model is provided with which local impact on costs, hospitalisations, and complications can be calculated. KEY POINTS:• Clinical practice guidelines recommend prophylactic intravenous hydration to prevent post-contrast adverse outcomes such as contrast-induced acute kidney injury. • Clinical practice guidelines have recently been updated, and standard prophylaxis is no longer recommended for the majority of patients. • The guideline updates have a large impact on daily clinical practice: relative reductions at Maastricht UMC+ were -?89% prophylaxis complications, -?93% hospitalisations, and -?91% costs, and similar reductions are expected for Dutch and adherent European medical centres.

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Hereditary diffuse gastric cancer: updated clinical practice guidelines.

Publications

Hereditary diffuse gastric cancer: updated clinical practice guidelines.

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