Surveillance of Individuals with a Family History of Pancreatic Cancer and Inherited Cancer Syndromes: A Strategy for Detecting Early Pancreatic Cancers.

Matsubayashi H1,2, Kiyozumi Y3, Ishiwatari H4, Uesaka K5, Kikuyama M6, Ono H7.

Author information

1: Division of Endoscopy, Shizuoka Cancer Center, Shizuoka 411-8777, Japan. h.matsubayashi@scchr.jp.
2: Division of Genetic Medicine Promotion, Shizuoka Cancer Center, Shizuoka 411-8777, Japan. h.matsubayashi@scchr.jp.
3: Division of Genetic Medicine Promotion, Shizuoka Cancer Center, Shizuoka 411-8777, Japan. y.kiyozumi@scchr.jp.
4: Division of Endoscopy, Shizuoka Cancer Center, Shizuoka 411-8777, Japan. h.ishiwatari@scchr.jp.
5: Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, Shizuoka 411-8777, Japan. k.uesaka@scchr.jp.
6: Department of Gastroenterology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo 113-0021, Japan. kikuyama110@yahoo.ne.jp.
7: Division of Endoscopy, Shizuoka Cancer Center, Shizuoka 411-8777, Japan. h.ono@scchr.jp.

Abstract

A family history of pancreatic cancer (PC) is a risk factor of PC, and risk levels increase as affected families grow in number and/or develop PC at younger ages. Familial pancreatic cancer (FPC) is defined as a client having at least two PC cases in a first degree relatives. In the narrow sense, FPC does not include some inherited cancer syndromes that are known to increase the risks of PC, such as Peutz-Jeghers syndrome (PJS), hereditary pancreatitis (HP), hereditary breast ovarian cancer syndrome (HBOC), and so on. FPC accounts for 5%-10% of total PC diagnoses and is marked by several features in genetic, epidemiological, and clinicopathological findings that are similar to or distinct from conventional PC. Recent advances in genetic medicine have led to an increased ability to identify germline variants of cancer-associated genes. To date, high-risk individuals (HRIs) in many developed countries, including FPC kindreds and inherited cancer syndromes, are screened clinically to detect and treat early-stage PC. This article highlights the concept of FPC and the most recent data on its detection.