Pancreatic lesions in von Hippel-Lindau syndrome: The coexistence of metastatic tumors from renal cell carcinoma and multiple cysts

Multiple cysts and benign cystadenomas of the pancreas have been documented occasionally in von Hippel-Lindau syndrome (HLS); however, the malignant i nvolvement of the pancreas in HLS is very rare. We report a case of HLS in which metastatic tumors from renal cell carcinoma (RCC) coexisted with mult iple cysts in the pancreas. A 22-year-old woman with a history of HLS had u ndergone a partial resection of the left kidney for RCC 3 Sears earlier, at which time a solid mass in the pancreatic tail and multiple pancreatic cys ts were also incidentally detected by computed tomography. Over the followi ng 3 years, the mass enlarged slightly, thus raising suspicions that it mig ht be a primary neoplasm of the pancreas. She was referred to the Departmen t of Surgery and Surgical Basic Science to undergo surgery. In addition to the tumor in the pancreatic tail, however, further tumors in the pancreatic head were also disclosed by preoperative celiac arteriography and intraope rative palpation and ultrasonography. A distal pancreatectomy was performed , because the enucleation of all the tumors in the pancreatic head was tech nically impossible and because the patient declined a total pancreatectomy. A histologic examination of the mass in the pancreatic tail revealed metas tatic RCC, This case emphasizes that metastatic disease should be included in the differential diagnosis when evaluating the pancreas in a patient wit h HLS.