Cysts containing renal cell carcinoma in von Hippel-Lindau disease

We report on a patient with von Hippel-Lindau disease. Exploration revealed 15 solid tumors and 18 cysts filled with clear fluid throughout both kidneys. All solid tumors consisted of clear cells consistent with renal cell carcinoma. All cysts were lined with similar clear cells. The solid tumors contained areas of clear cells surrounding small cystic spaces and the large cysts appeared to be an exaggeration of this phenomenon. There is a place for radical ablative surgery in patients with multiple renal cell carcinomas in von Hippel-Landau disease. Unroofing and gross examination of apparently simple cysts may not be adequate to rule out the presence of carcinoma in these patients.