Management of renal cell carcinoma in von Hippel-Lindau disease

Background An evaluation of nephron-sparing surgery (NSS) or radical nephre ctomy (RN) for treating renal cell carcinoma (RCC) in patients with von Hip pel-Lindau disease (VEFL) was carried out. Methods Between 1976 and 1997, 10 patients with RCC from four VHL families, of whom seven were from one family, were studied by clinical and histopath ological examination. Before 1991, three patients were treated using RN, an d thereafter five patients were treated using NSS. Two patients were not op erated on. Results RCCs in our patients showed a slow growth rate (on average 0.3 cm y ear(-1)), and asymptomatic patients presented with tumours of low-grade mal ignancy. In all patients, tumours were surrounded by a fibrous pseudocapsul e. In 5 out of 17 rumours, pseudocapsular invasion was observed, and three of these five tumours broke through the pseudocapsule. To date, these patie nts have not shown a less favourable outcome than those without pseudocapsu lar involvement by tumour growth. Multicentricity of RCC was relatively low (4.6 lesions per kidney). In two of the three RN patients, only a single s atellite lesion, in the direct vicinity of a RCC, was found in one kidney. Six tumours (1.8-5.5 cm) were enucleated by NSS. During a mean follow-up of 30 months, renal function in these patients was well preserved. Conclusions In our patients, RCCs grew slowly, mere of low grade, had a den se fibrous pseudocapsule and were thus good candidates for NSS.