Combination of right nephrectomy and total pancreaticoduodenectomy for Von Hippel-Lindau disease.
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Arkadopoulos
Nikolaos
N
Second Department of Surgery, Aretaieion Hospital, Athens University School of Medicine. Athens, Greece. narkado@otenet.gr
Karapanos
Konstantinos
K
Stafyla
Vaia
V
Yiallourou
Anneza
A
Koureas
Andreas
A
Kondi-Pafiti
Agathi
A
Smyrniotis
Vassilios
V
D000236:Adenoma / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D002292:Carcinoma, Renal Cell / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D009392:Nephrectomy / Q000379:methods*; D010190:Pancreatic Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D016577:Pancreaticoduodenectomy / Q000379:methods*; D013156:Splenectomy; D014057:Tomography, X-Ray Computed; D006623:von Hippel-Lindau Disease / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*