Department of Visceral-, Thoracic- and Vascular Surgery, Philipps-University Marburg, Baldingerstrasse, 35043, Marburg, Germany.
Fam Cancer. 2019 Jul;18(3):359-362. doi: 10.1007/s10689-019-00125-9.
A hereditary predisposition to pancreatic ductal adenocarcinoma (PDAC) is reported in approximately 5% of patients. Familial pancreatic cancer (FPC) accounts for the vast majority of hereditary PDAC cases. FPC defines families with two or more affected first-degree relatives with PDAC that do not fulfil the criteria of any other inherited tumor syndrome or families with PDAC in three or more relatives of any degree. The current progress report focuses on the knowledge of FPC with regard to molecular pathogenesis, clinical and molecular diagnosis, surveillance and novel treatment options reported in the last 5 years.
遗传性胰腺导管腺癌（PDAC）易感性约占患者的 5%。家族性胰腺癌（FPC）占遗传性 PDAC 病例的绝大多数。FPC 定义为具有两个或更多受影响的一级亲属患有 PDAC 且不符合任何其他遗传性肿瘤综合征标准的家族，或具有三个或更多任何程度亲属的 PDAC 的家族。本进展报告重点介绍了过去 5 年来关于 FPC 的分子发病机制、临床和分子诊断、监测和新的治疗选择的知识。
