Combination of right nephrectomy and total pancreaticoduodenectomy for von hippel-lindau disease
Date
2010Author
Arkadopoulos, NikolaosKarapanos, Konstantinos
Stafyla, V. K.
Yiallourou, Anneza I.
Koureas, Andreas
Kondi-Pafiti, Agathi
Smyrniotis, V.
Source
Journal of the PancreasVolume
11Issue
3Pages
270-272Google Scholar check
Keyword(s):
Metadata
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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.