Gut Liver.  2019 Sep;13(5):498-505. 10.5009/gnl18449.

Familial Pancreatic Cancer and Surveillance of High-Risk Individuals

Affiliations
  • 1Division of Genetic Medicine Promotion, Shizuoka Cancer Center, Shizuoka, Japan. h.matsubayashi@scchr.jp
  • 2Division of Endoscopy, Shizuoka Cancer Center, Shizuoka, Japan.
  • 3Department of Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan.
  • 4Division of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan.

Abstract

Family history of pancreatic cancer (PC) is a risk factor for PC development, and the risk level correlates with the number of affected families. A case of PC with ≥1 PC cases in the first-degree relative is broadly defined as familial pancreatic cancer (FPC) and accounts for 5% to 10% of total PC cases. FPC possesses several epidemiological, genetic and clinicopathological aspects that are distinct from those of conventional PCs. In Western countries, FPC registries have been established since the 1990s, and high-risk individuals are screened to detect early PCs. For the pharmacotherapy of FPC, especially in cases with germline pathogenic BRCA mutations, regimens using platinum and poly (ADP-ribose) polymerase inhibitor have recently been studied for their effectiveness. To date, the concept of FPC has prevailed in Western countries, and it has begun to infiltrate into Eastern countries. As the genetic background and environmental conditions vary in association with ethnicity and living area, we need to establish our own FPC registries and accumulate data in Asian countries.

Keyword

Familial pancreatic cancer; High risk; Genetic; Surveillance; Treatment

MeSH Terms

Asian Continental Ancestry Group
Drug Therapy
Genetic Background
Humans
Pancreatic Neoplasms*
Platinum
Registries
Risk Factors
Platinum
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