[Renal and adrenal involvement in von Hippel-Lindau disease: clinical features and therapeutic strategies].
OBJECTIVE
We report our experience of renal and/or adrenal manifestations of von Hippel-Lindau disease and propose a practical approach.


METHODS
Eight patients (mean age: 43 years) presented with predominant renal and adrenal lesions in 6 cases and 2 cases, respectively.


RESULTS
All patients are alive with a mean follow-up of 8.1 years. A local recurrence after partial nephrectomy was observed in two cases. Two patients are in renal failure and are treated by dialysis and two patients require hormone replacement therapy for adrenal insufficiency.


CONCLUSIONS
The predegenerative nature of simple renal was not observed. Conservative renal surgery is adapted to small renal tumours, with a low cytological grade and without any distant lesions in the same kidney. Radical nephrectomy is reserved for large lesions (greater than 5 cm) with a high cytological grade. The presence of pheochromocytoma must be systematically excluded. Preservation of the adrenal gland in the case of homolateral renal surgery for cancer is recommended. The reliability of the genetic test allows early diagnosis of this disease.
Chassagne
S
S
Service d'Urologie et Chirurgie de la Transplantation, Hôpital Edouard Herriot, Lyon, France.
Martin
X
X
Cloix
P
P
Dawahra
M
M
Bret
P
P
Giraud
S
S
Fendler
J P
JP
Dubernard
J M
JM
D000307:Adrenal Gland Diseases / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D000328:Adult; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D010673:Pheochromocytoma / Q000209:etiology; D006623:von Hippel-Lindau Disease / Q000150:complications*
Atteintes rénales et surrénaliennes dans la maladie de von Hippel-Lindau: aspects cliniques et stratégies thérapeutiques.