Pathological features of total gastrectomy specimens from asymptomatic hereditary diffuse gastric cancer patients and implications for clinical management

Histopathology
João P RochaFátima Carneiro

Abstract

Hereditary diffuse gastric cancer (HDGC) is an autosomal dominant syndrome characterised by multigenerational diffuse gastric cancer, and is mainly caused by germline alterations in the CDH1 gene. Currently, endoscopy has limited diagnostic accuracy, and total gastrectomy (TG) is the treatment of choice for asymptomatic CDH1 carriers. In this study, we aimed to obtain a better understanding of HDGC syndrome by exploring the histopathological findings of TG specimens from asymptomatic HDGC patients. A comprehensive literature review was carried out, searching for TGs performed in asymptomatic HDGC patients. Fourteen unpublished cases, analysed in our institution, were also included. The series encompassed 174 CDH1 carriers. Preoperative endoscopic biopsies were positive in 28.3%. A macroscopic lesion was apparent in 11.7% of TGs. Histopathological analysis revealed intraepithelial lesions and/or intramucosal signet ring cell carcinoma in 87.9% of TGs. When we explored the type of protocol used for handling the specimens, we found that microscopic cancer foci were detected in 95.3% of TGs when a total-embedding protocol (assessment of the totality of gastric mucosa) was applied, and only in 62.5% when no specific protocol was use...Continue Reading

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Citations

Apr 17, 2019·Familial Cancer·Rachel S van der PostFátima Carneiro
May 5, 2020·BMC Gastroenterology·Elisabeth NiemeyerAlexander E Volk
Jan 1, 2021·Histopathology·Irene GulloFátima Carneiro
Nov 21, 2020·Annual Review of Medicine·Nastazja Dagny PilonisMassimiliano di Pietro
Jun 8, 2021·Journal of Laparoendoscopic & Advanced Surgical Techniques. Part a·Javier Ithurralde-ArgerichFederico Cuenca-Abente

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