PANCREATIC LESIONS IN VON HIPPEL-LINDAU DISEASE - PREVALENCE, CLINICAL-SIGNIFICANCE, AND CT FINDINGS

OBJECTIVE. The purposes of this study were to determine the nature, pr evalence, and CT findings of pancreatic lesions in patients with van H ippel-Lindau disease and to determine whether identification of pancre atic cysts and neoplasms is important in establishing the diagnosis of van Hippel-Lindau disease. SUBJECTS AND METHODS. The medical records and radiologic images of 52 patients with van Hippel-Lindau disease wh o were evaluated at our institution between 1976 and 1992, and who at some stage underwent abdominal CT, sonography, or MR imaging, were rev iewed. The nature, prevalence, and CT findings of the pancreatic lesio ns were determined, and the role of the pancreatic abnormalities in es tablishing the diagnosis of von Hippel-Lindau disease was studied. RES ULTS. Twenty-nine (56%) of the 52 patients had pancreatic lesions. Nin eteen patients had pancreatic cysts and no other pancreatic lesion. Fo ur patients had islet cell tumors only, one had a microcystic adenoma only, and three had indeterminate pancreatic masses. One patient had c ysts and an islet cell tumor, and another patient had cysts, an islet cell tumor, and a microcystic adenoma. In six patients (12%), pancreat ic lesions were the only abdominal manifestation of van Hippel-Lindau disease. In three patients screened because of a family history of van Hippel-Lindau disease, no CNS abnormalities were present, and the onl y abdominal lesions were in the pancreas (cysts in two cases, islet ce ll carcinoma in the other). Thus, the pancreatic lesion was an importa nt factor in establishing a diagnosis of van Hippel-Lindau disease in these patients.CONCLUSION. Pancreatic lesions may be the only abdomina l manifestation of von Hippel-Lindau disease. CT findings include cyst s, islet cell tumors, and microcystic adenomas. Pancreatic lesions, in cluding cysts, may precede any other manifestation of von Hippel-Linda u disease by several years, and recognition permits earlier diagnosis in patients being screened for van Hippel-Lindau disease.