Peutz-Jeghers syndrome (PJS) is a rare disorder known to be associated with an increased cancer risk, including pancreatic cancer. Surveillance of the pancreas might improve the poor prognosis of pancreatic cancer. In this study we determined the risk for pancreatic cancer and cancer in the pancreatic region (including biliary cancer) in patients with PJS. The relative risk for pancreatic cancer in PJS was 76 in comparison with the general population, and 96 for cancer in the pancreatic region.  We recommend pancreatic surveillance in PJS patients from the age of 30 years. As long as there is no proof of benefit of such a surveillance program, this should be done in a research setting. (By Drs. S.E. Korsse, http://jmg.bmj.com/content/50/1/59 )