Bilateral renal cell carcinoma associated with von Hippel-Lindau disease

A 52-year-old man was referred to the department of neurosurgery of our hospital for evaluation of left occipital pain on September 27, 1982. Brain CT and arteriography showed midcerebellar tumor and an operation was performed on October 7, 1982. Histopathologically, the tumor was hemangioblastoma (solid type). His whole-body CT showed bilateral multiple renal tumors but no angioma retinae were found. Angiography revealed that the lesions were bilateral multiple (more than 20) renal tumors, bilateral adrenal tumors and left retroaortic renal vein. The patient underwent bilateral radical nephrectomy and lymphadenectomy on December 15, 1982. Renal cell carcinoma (grade II greater than III) with adrenal and left renal vein involvement were noted on the pathologic specimen. Postoperatively, he received supplement therapy with hydrocortisone and hemodialysis, but died of bleeding from the recurrent hemangioblastoma on July 7, 1983.