Renal Malignancy in von Hippel-Lindau's Disease

Abstract

We present our experience with 4 patients with von Hippel-Lindau's disease. They all had asymptomatic renal malignancy, and three patients had bilateral tumors. None of the patients had metastases from their renal tumors, but two patients had developed new tumors which demanded surgery. Abdominal computerized tomography was first choice in the initial evaluation, eventually supplemented by renal angiography. The treatment should be conservative, but bilateral nephrectomy and hemodialysis may be required. Screening of at-risk family members and close and lifelong follow-up is emphasized.

Keywords:

• Von Hippel-Lindau's disease
• renal cell carcinoma