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.

• Authors: Nikolaos Arkadopoulos, Konstantinos Karapanos, Vaia Stafyla, Anneza Yiallourou, Andreas Koureas, Agathi Kondi-Pafiti, Vassilios Smyrniotis
• Journal: JOP : Journal of the pancreas (JOP) Vol. 11 Issue 3 Pg. 270-2 (May 05 2010) ISSN: 1590-8577 [Electronic] Italy
• PMID: 20442526 (Publication Type: Case Reports, Journal Article)

Topics

• Adenoma (diagnostic imaging, pathology, surgery)
• Carcinoma, Renal Cell (diagnostic imaging, pathology, surgery)
• Female
• Humans
• Magnetic Resonance Imaging
• Middle Aged
• Neoplasms, Multiple Primary (diagnostic imaging, pathology, surgery)
• Nephrectomy (methods)
• Pancreatic Neoplasms (diagnostic imaging, pathology, surgery)
• Pancreaticoduodenectomy (methods)
• Splenectomy
• Tomography, X-Ray Computed
• von Hippel-Lindau Disease (diagnostic imaging, pathology, surgery)