About this Journal Submit a Manuscript Table of Contents
BioMed Research International
Volume 2013 (2013), Article ID 385132, 8 pages
http://dx.doi.org/10.1155/2013/385132
Research Article

History, Pathogenesis, and Management of Familial Gastric Cancer: Original Study of John XXIII's Family

1Department of Human Pathology and Oncology, Section of General Surgery and Surgical Oncology and Translational Research Laboratory, University of Siena, Viale Bracci, 53100 Siena, Italy
2Azienda Sanitaria Locale, Bergamo Province, Via alla Guardina 24039, Sotto il Monte Giovanni XXIII, Italy
3Medical Faculty of the University of Porto, st. John Hospital Center and Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), rua dr. Roberto Frias 4200-465 Porto, Portugal

Received 23 October 2012; Accepted 21 November 2012

Academic Editor: Kathleen Claes

Copyright © 2013 Giovanni Corso et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background. Hereditary diffuse gastric cancer is associated with the E-cadherin germline mutations, but genetic determinants have not been identified for familial intestinal gastric carcinoma. The guidelines for hereditary diffuse gastric cancer are clearly established; however, there are no defined recommendations for the management of familial intestinal gastric carcinoma. Methods. In this study we describe Pope John XXIII's pedigree that harboured gastric cancer as well as six other family members. Family history was analysed according to the International Gastric Cancer Linkage Consortium criteria, and gastric tumours were classified in accord with the last Japanese guidelines. Results. Seven out of 109 members in this pedigree harboured gastric cancer, affecting two consecutive generations. John XXIII's clinical tumour (cTN) was classified as cT4bN3a (IV stage). In two other cases, gastric carcinomas were classified as intestinal histotype and staged as pT1bN0 and pT2N2, respectively. Conclusions. Pope John XXIII's family presents a strong aggregation for gastric cancer affecting almost seven members; it spreads through two consecutive generations. In absence of defined genetic causes and considering the increased risk of gastric cancer’s development in these families, as well as the high mortality rates and advanced stages, we propose an intensive surveillance protocol for asymptomatic members.