[Treatment of von Hippel-Lindau disease with renal involvement]

To try to define when and what type of operation should be performed in von Hippel-Lindau disease (VHL) with renal involvement.

Between 1985 and July 1996, 43 patients with renal involvement of VHL disease were followed in our department. We decided to operate on patients with one or several solid or complex tumours greater than 2.5 cm in diameter, by means of conservative surgery (tumour excision removing a layer of healthy parenchyma) when the kidney did not contain more than 5 or 6 tumours or radical nephrectomy in the other cases. Subsequent follow-up consisted of CT, chest x-ray and renal function tests every 6 months.

Twenty-nine patients with a mean age of 33.6 years were operated. 21 patients (29 kidneys) were treated by conservative surgery: in situ in 20 cases and ex vivo in 9 cases; 3 cases were treated by bilateral radical nephrectomy, 4 by unilateral radical nephrectomy and 1 by resection of the prominent dome for urinary tract obstruction. Four cases of acute arterial thrombosis were observed following ex vivo surgery, requiring nephrectomy in every case. No serious complications were observed with in situ conservative surgery. A new lesion in the renal parenchyma remaining after conservative surgery was observed in 5 cases during follow-up (mean: 29 months). No local recurrence was observed after radical nephrectomy. Two nonoperated patients developed metastatic disease.

Conservative treatment is usually possible: either simple surveillance, in the absence of any solid or complex tumours larger than 2.5 cm in diameter, or conservative surgery for tumours larger than 2.5 cm in diameter, with no more than 6 tumours per kidney; this attitude requires six-monthly follow-up by CT of the renal parenchyma.