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Clinical Trial
. 2013:2013:385132.
doi: 10.1155/2013/385132. Epub 2012 Dec 26.

History, pathogenesis, and management of familial gastric cancer: original study of John XXIII's family

Giovanni Corso  1 , Fabrizio Roncalli, Daniele Marrelli, Fátima Carneiro, Franco Roviello
Affiliations
Clinical Trial

History, pathogenesis, and management of familial gastric cancer: original study of John XXIII's family

Giovanni Corso et al. Biomed Res Int. 2013.

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.

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Figures

Figure 1
Figure 1
(a) Schematic pedigree of John XXIII's family with seven cases affected by primary gastric carcinoma (generations IV and V). Clinicopathological information were available for cases marked with underline numbers; (b) Roncalli's original pedigree, firstly described in 1968. The bold characters indicated members affected by primary gastric tumours; the proband was indicated with the papal shield.
Figure 2
Figure 2
Coronation day, November 1958. Pope John XXIII with pontiff vestments wearing the papal tiara and “fanon” that defines the supreme authority as Roman Pontiff of the Catholic Church.
Figure 3
Figure 3
The proposed flow chart is suggested for the gastric surveillance in asymptomatic members recorded in this pedigree and in cases with familial intestinal gastric cancer. Some indications, such as the age for the first gastroendoscopy, are specific for this pedigree.
Figure 4
Figure 4
Representative sample of perforated gastric tumour (personal archive); arrow indicates the depth perforation.
See this image and copyright information in PMC

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