How to manage cystic tumors of the pancreas in high-risk individuals

Abstract
Cystic tumors of the pancreas are detected with increasing frequency. Reliable differentiation between benign cysts, cysts with malignant potential, and cysts that harbor invasive cancer is crucial for patient management, but poses a clinical challenge. Interestingly, in individuals with an increased risk for pancreatic cancer, the prevalence of pancreatic cysts is high, up to 42 %. Cyst management in these high-risk individuals presents an even greater challenge, as little is known about the natural behavior of cystic lesions in general, but even less in this high-risk group.

Two groups with a hereditary risk for pancreatic cancer are recognized: individuals with a cancer-prone gene mutation and those without, but who have a strong family history of pancreatic cancer. The Cancer of the Pancreas Screening (CAPS) Consortium recommends screening individuals with an estimated tenfold increased risk on developing pancreatic cancer. Their guidelines recommend annual endoscopic ultrasonography and MRI/MRCP. Preliminary studies show an overall diagnostic yield of 9 %, but data on the long-term outcome of this surveillance strategy are still lacking. For the management of cystic tumors of the pancreas, the revised Sendai criteria are generally applied, recommending regular follow-up by EUS or MRI.

This chapter provides an overview of the current knowledge on cystic tumors of the pancreas in individuals with a hereditary risk for developing pancreatic cancer and discusses cyst management in these high-risk individuals.