Peutz–Jeghers syndrome and screening for pancreatic cancer

Abstract

Abstract 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.