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Management of pancreatic lesions in von Hippel-Lindau disease.
- Source :
-
World journal of surgery [World J Surg] 1997 Mar-Apr; Vol. 21 (3), pp. 307-12. - Publication Year :
- 1997
-
Abstract
- 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.
Details
- Language :
- English
- ISSN :
- 0364-2313
- Volume :
- 21
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- World journal of surgery
- Publication Type :
- Academic Journal
- Accession number :
- 9015176
- Full Text :
- https://doi.org/10.1007/s002689900234