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Hereditary diffuse gastric cancer: updated clinical practice guidelines
- Source :
- Lancet Oncology, 21, 8, pp. e386-e397, Lancet Oncol, Lancet Oncology, 21, e386-e397
- Publication Year :
- 2020
-
Abstract
- Contains fulltext : 225261.pdf (Publisher’s version ) (Closed access) 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.
- Subjects :
- 0301 basic medicine
Oncology
medicine.medical_specialty
medicine.medical_treatment
Lobular carcinoma
Article
Cancer syndrome
03 medical and health sciences
0302 clinical medicine
Breast cancer
Germline mutation
Neoplastic Syndromes, Hereditary
Stomach Neoplasms
Internal medicine
medicine
Tumours of the digestive tract Radboud Institute for Molecular Life Sciences [Radboudumc 14]
Humans
Genetic testing
medicine.diagnostic_test
business.industry
Cancer
medicine.disease
030104 developmental biology
030220 oncology & carcinogenesis
Gastrectomy
Hereditary diffuse gastric cancer
business
Subjects
Details
- ISSN :
- 14702045
- Volume :
- 21
- Database :
- OpenAIRE
- Journal :
- Lancet Oncology
- Accession number :
- edsair.doi.dedup.....e170c9ddf442b6b43660788219059b7b