Huge pancreas helps diagnosis in von Hippel-Lindau disease.

We describe five patients diagnosed with von Hippel-Lindau disease who complained of abdominal distension, pain and discomfort for a long time. All patients underwent ultrasonography, CT scan and MRI, which showed huge pancreas filled with multiple cysts. Additionally, extrapancreatic findings such as cerebellar hemangioblastoma (3 patients), retinal hemangioblastoma (2), renal cell carcinoma (3), renal adenoma (1), renal cysts (4), and splenic cyst (1) helped to reach the right diagnosis. One patient who had no known associated pathology had a family history of von Hippel-Lindau disease. Pancreatic cysts detected on imaging may be a clue to the diagnosis of von Hippel-Lindau disease. In all patients with multiple pancreatic cysts, this disease should be included in the differential diagnosis.