Pancreatic lesions in von Hippel-Lindau disease: Prevalence, clinical significance, and CT findings

OBJECTIVE. The purposes of this study were to determine the nature, prevalence, and CT findings of pancreatic lesions in patients with von Hippel-Lindau disease and to determine whether identification of pancreatic cysts and neoplasms is important in establishing the diagnosis of von Hippel- Lindau disease. SUBJECTS AND METHODS. The medical records and radiologic images of 52 patients with von Hippel-Lindau disease who were evaluated at our institution between 1976 and 1992, and who at some stage underwent abdominal CT, sonography, or MR imaging, were reviewed. The nature, prevalence, and CT findings of the pancreatic lesions were determined, and the role of the pancreatic abnormalities in establishing the diagnosis of von Hippel-Lindau disease was studied. RESULTS. Twenty-nine (56%) of the 52 patients had pancreatic lesions. Nineteen patients had pancreatic cysts and no other pancreatic lesion. Four patients had islet cell tumors only, one had a microcystic adenoma only, and three had indeterminate pancreatic masses. One patient had cysts and an islet cell tumor, and another patient had cysts, an islet cell tumor, and a microcystic adenoma. In six patients (12%), pancreatic lesions were the only abdominal manifestation of von Hippel- Lindau disease. In three patients screened because of a family history of von Hippel-Lindau disease, no CNS abnormalities were present, and the only abdominal lesions were in the pancreas (cysts in two cases, islet cell carcinoma in the other). Thus, the pancreatic lesion was an important factor in establishing a diagnosis of von Hippel-Lindau disease in these patients. CONCLUSION. Pancreatic lesions may be the only abdominal manifestation of von Hippel-Lindau disease. CT findings include cysts, islet cell tumors, and microcystic adenomas. Pancreatic lesions, including cysts, may precede any other manifestation of von Hippel-Lindau disease by several years, and recognition permits earlier diagnosis in patients being screened for von Hippel-Lindau disease.