Risk-reducing total gastrectomy in asymptomaticCDH1carriers Experience of a tertiary hospital

• Type: Article in International Scientific Journal
• Date: 2020

Authors

Devezas, V (Author) Other; Baptista, M (Author) Other; Irene Gullo (Author) FMUP; Rocha, J (Author) Other; Sousa, F (Author) Other; Wen, XG (Author) Other; Preto, J (Author) Other; Costa, S (Author) Other; Castedo, S (Author) Other; Garrido, L (Author) Other; Oliveira, C (Author) Other; Laura Barbosa (Author) FMUP; Carneiro F (Author) FMUP; Barbosa, J (Author) Other

Journal

Title: European Surgery - Acta Chirurgica Austriaca

Vol. 52

Pages: 171-178

ISSN: 1682-8631

Publisher: Springer Nature

Indexing

ISI Web of Knowledge - 3 Citations

Scopus - 3 Citations

Other information

Authenticus ID: P-00R-WP6

DOI: 10.1007/s10353-020-00630-7

Abstract (EN): Background Hereditary diffuse gastric cancer is an autosomal dominant cancer susceptibility syndrome characterized by increased risk for gastric cancer and lobular breast cancer and mainly caused by germline alterations in theCDH1gene. Methods This was an observational, retrospective, and unicentric study. Clinical data from patients with hereditary diffuse gastric cancer who had risk-reducing total gastrectomy between June 2005 and January 2018 were collected. A thorough histopathological analysis of gastrectomy specimens was performed, according to the current recommendations of the International Gastric Cancer Linkage Consortium. Results In 59 families fulfilling the clinical criteria forCDH1testing, we identified the sameCDH1missense pathogenic variant in five families. Thirty-nineCDH1carriers were identified. FourCDH1carriers died with advanced diffuse gastric cancer, 19 chose risk-reducing total gastrectomy, and 16 opted for endoscopic surveillance, despite being duly informed of the limited utility of periodic gastric endoscopy in this context. The 19 asymptomaticCDH1carriers undergoing surgery had an age range of 14-63 years (median, 39 years) and the median hospital stay was 6 days. Laparoscopic total gastrectomy was performed on four of 19CDH1carriers. There were no cases of mortality or major morbidity in this procedure. The range of weight loss in our series was 13%. Histopathological examination of the entirety of gastric mucosa revealed intramucosal gastric carcinoma foci (pT1a) in 17 of 19 (89.5%) total gastrectomy specimens. No lymph node metastases were detected (pN0). Conclusion Risk-reducing total gastrectomy remains the best clinical management for patients with hereditary diffuse gastric cancer harboring a germline, pathogenicCDH1variant. In our series, intramucosal carcinoma foci were found in the great majority (89.5%) ofCDH1carriers.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 8