Familial pancreatic cancer (FPC) is a rare, hereditary tumour syndrome. High morbidity and mortality is associated with development of pancreatic cancer in these high-risk individuals and, as outlined in this Review, screening of FPC families seems appropriate. Here, Bartsch and colleagues describe the current knowledge of FPC, including phenotype, underlying genetic causes and clinical management (including genetic counselling and screening).
- Detlef K. Bartsch
- Thomas M. Gress
- Peter Langer