Background:
Cancer risk, including pancreatic, is high in those with Peutz–Jeghers syndrome (PJS). It has been suggested that such patients should undergo screening for pancreatic cancer.

Methods:
The risk of pancreatic cancer in PJS, pancreatic screening and potential screening strategies were reviewed. Cost-effectiveness was assessed according to American Gastroenterology Association guidelines and a risk stratification model proposed by the European Registry of Hereditary Pancreatitis and Familial Pancreatic Cancer.

Results:
The risk of pancreatic cancer is increased in PJS but screening would cost over US $350 000 per life saved. Risk stratification reduces cost by $100 000 and costs fall to $50 000 per life saved if deaths from other forms of cancer are avoided.

Conclusion:
Screening should be performed only on a research basis to evaluate the benefit and cost-effectiveness in high-risk groups