Department of Human Pathology and Oncology, Section of General Surgery and Surgical Oncology and Translational Research Laboratory, University of Siena, Viale Bracci, 53100 Siena, Italy.
Biomed Res Int. 2013;2013:385132. doi: 10.1155/2013/385132. Epub 2012 Dec 26.
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.
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.
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.
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.
遗传性弥漫型胃癌与 E-钙黏蛋白种系突变有关，但家族性肠型胃癌的遗传决定因素尚未确定。遗传性弥漫型胃癌的指南已经明确制定；然而，对于家族性肠型胃癌的管理尚无明确的建议。
在本研究中，我们描述了教皇约翰二十三世的家族系谱，其中包括胃癌以及其他六名家族成员。家族史按照国际胃癌连锁研究协会的标准进行分析，胃肿瘤的分类符合日本最新的指南。
该家族系谱中的 109 名成员中有 7 人患有胃癌，影响了两代人。约翰二十三世的临床肿瘤（cTN）被归类为 cT4bN3a（IV 期）。在另外两例中，胃癌被归类为肠型，分期分别为 pT1bN0 和 pT2N2。
教皇约翰二十三世的家族中，有近 7 名成员强烈聚集患有胃癌，且通过两代人传播。由于这些家族中胃癌发生的风险增加，以及高死亡率和晚期阶段，在没有明确遗传原因的情况下，我们建议对无症状成员进行强化监测。
