Management of pancreatic lesions in von Hippel-Lindau disease

Twelve patients with von Hippel-Lindau disease were collected in our institute from 1981 to 1995. All had a family history of the disease. Eleven patients underwent abdominal computed tomography, sonography, or angiographic studies. Ten had pancreatic involvement that included cystic lesions in nine and a solid lesion in one. Seven patients were asymptomatic. Another three presented with obstructive jaundice or upper gastrointestinal (UGI) bleeding. Except for case 8, who died of a central nervous system complication soon after diagnosis of the pancreatic lesion, the other patients had been found to have pancreatic involvement for a variable period of time, ranging from 1 to 13 years (median 5 years). Serous cystadenoma was proved pathologically in two with cystic lesions, and pancreatic endocrine tumor was diagnosed in one with a solid mass. One patient (case 1) underwent biliary bypass due to obstructive jaundice and died of cholangitis and pneumonia 6 years later. One patient (case 3) had total pancreatectomy and lived well with good diabetic control for more than 5 years. The patient with a solid lesion was explored because of repeated UGI bleeding. Surgical resection was impossible owing to advanced tumor with vascular involvement, and a pancreatic endocrine tumor was diagnosed pathologically. He was followed for 1 year. The other seven patients remained asymptomatic during the successive follow-up period. From a literature review and our own experience, we suggest that conservative measures are adequate for the cystic lesions; however, aggressive resection is mandatory for a solid pancreatic lesion in von Hippel-Lindau disease.