Patients with von Hippel–Lindau (VHL) disease are susceptible to a range of neoplasms. More than 50% of these patients suffer from a range of pancreatic lesions at some point in their lifetime, and the different types of lesions require different management approaches. In this Review, the authors describe the types of pancreatic lesions that are associated with VHL disease, and discuss the management of patients with different pancreatic manifestations; an overall patient-management algorithm is also presented.
- Xavier M. Keutgen
- Pascal Hammel
- Electron Kebebew
