[Hereditary gastric cancer syndromes and their association with specific histological subtypes]. / Prédispositions génétiques au cancer gastrique et leur association au type histologique.
Bull Cancer
; 110(5): 512-520, 2023 May.
Article
em Fr
| MEDLINE
| ID: mdl-35963792
ABSTRACT
About 5% of gastric cancers are associated with hereditary cancer syndromes. Histology is paramount in this context, as major susceptibility genes are associated with specific subtypes. Germline pathogenic variants in CDH1 and CTNNA1 cause Hereditary Diffuse Gastric Cancer (HDGC). Major advances have been made in the past ten years regarding HDGC. Penetrance estimates for diffuse cancer are now lower than previously thought, at 30-40%. Surveillance upper gastrointestinal endoscopy is now an acceptable alternative to prophylactic total gastrectomy. Indeed, its sensitivity in detecting advanced disease is satisfactory assuming it is performed by an expert and according to a specific protocol. The risk of intestinal-type gastric cancer is increased in patients with Lynch syndrome, although it is much lower than the risk of colorectal and endometrial cancer. Intestinal-type gastric cancers are also observed in excess in patients with hereditary polyposis, the main one being APC-associated familial adenomatous polyposis. The main and most clinically relevant manifestations in patients with polyposes remain colorectal and duodenal polyps and carcinomas, well ahead of gastric cancer. Finally, recent data point towards increased gastric cancer risk in hereditary breast and ovarian cancer.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Gástricas
/
Síndromes Neoplásicas Hereditárias
/
Neoplasias Colorretais Hereditárias sem Polipose
/
Polipose Adenomatosa do Colo
Tipo de estudo:
Guideline
/
Risk_factors_studies
Limite:
Humans
Idioma:
Fr
Ano de publicação:
2023
Tipo de documento:
Article