Combination of Right Nephrectomy and Total Pancreaticoduodenectomy for von Hippel-Lindau Disease
Abstract
Context Von Hippel-Lindau disease is an inherited syndrome of multiorgan neoplasia caused by a germline mutation in the von Hippel-Lindau gene and can include central nervous system tumors, renal cell carcinomas and benign pancreatic cystic tumors. Case report We report the case of a 56-year-old patient who had a past history of cerebellar hemangioblastoma and presented with abdominal pain. Imaging revealed renal tumors and multiple pancreatic tumors which caused duodenal and pancreatic duct compression. The patient was treated with a combination of radical right nephrectomy, total pancreaticoduodenectomy and splenectomy. Pathology identified a multifocal unilateral clear cell renal carcinoma which interestingly coexisted with multiple large pancreatic serous microcystic adenomas with infiltration of the fibrous capsule. Conclusion In past cases of von Hippel-Lindau disease, pancreatic adenomas with malignant transformation have not been reported. In our case, the infiltration of the fibrous capsule by parenchymal cells may indicate malignant transformation.
Image: Section of serous microcystic adenoma in the pancreatic head.
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References
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Copyright (c) 2010 Nikolaos Arkadopoulos, Konstantinos Karapanos, Vaia Stafyla, Anneza Yiallourou, Andreas Koureas, Agathi Kondi-Pafiti, Vassilios Smyrniotis
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