Management of renal cell carcinoma in von Hippel-Lindau disease.
BACKGROUND
An evaluation of nephron-sparing surgery (NSS) or radical nephrectomy (RN) for treating renal cell carcinoma (RCC) in patients with von Hippel-Lindau disease (VHL) 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 histopathological examination. Before 1991, three patients were treated using RN, and thereafter five patients were treated using NSS. Two patients were not operated on.


RESULTS
RCCs in our patients showed a slow growth rate (on average 0.3 cm year-1), and asymptomatic patients presented with tumours of low-grade malignancy. In all patients, tumours were surrounded by a fibrous pseudocapsule. In 5 out of 17 tumours, pseudocapsular invasion was observed, and three of these five tumours broke through the pseudocapsule. To date, these patients have not shown a less favourable outcome than those without pseudocapsular 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 satellite 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, were of low grade, had a dense fibrous pseudocapsule and were thus good candidates for NSS.
Hes
F J
FJ
Department of Internal Medicine, University Hospital, Utrecht, The Netherlands.
Slootweg
P J
PJ
van Vroonhoven
T J
TJ
Hené
R J
RJ
Feldberg
M A
MA
Zewald
R A
RA
Ploos van Amstel
J K
JK
Höppener
J W
JW
Pearson
P L
PL
Lips
C J
CJ
D000328:Adult; D017668:Age of Onset; D002292:Carcinoma, Renal Cell / Q000150:complications / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D002353:Carrier State; D019468:Disease Management; D005260:Female; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D009392:Nephrectomy; D010375:Pedigree; D012189:Retrospective Studies; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000235:genetics / Q000401:mortality / Q000601:surgery*