Diagnosis: Renal cell carcinoma in von Hippel-Lindau disease.

            Comments: This patient with von Hippel-Lindau disease had a multifocal conventional renal cell carcinoma. There were a total of 5 nodules: 4 nodules of clear cell carcinoma, Fuhrman 1-2 (Figs. 1 and 2) and 1 nodule of eosinophilic cells, Fuhrman 3 (Figs. 3 and 4). There were also neoplastic cysts (Fig. 5) as well as simple cysts. The neoplasia was confined to the kidney. Renal tumors in von Hippel-Lindau disease tend to be multifocal and bilateral.