PTU - Polskie Towarzystwo Urologiczne
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NEPHRON-SPARING SURGERY FOR RENAL CELL CARCINOMA IN VON HIPPEL-LINDAU DISEASE
Article published in Urologia Polska 1998/51/2.

authors

Marcin Słojewski 1, Bartłomiej Gliniewicz 1, Andrzej Sikorski 1, Marek Grabowski 1, Karol Krzystolik 2, Cezary Cybulski 2, Jan Lubiński 2
1 Klinika Urologii PAM w Szczecinie
Kierownik Kliniki: prof. dr hab. med. A. Sikorski
2 Zakład Genetyki i Patomorfologii PAM w Szczecinie
Kierownik Zakładu: prof. dr hab. med. J. Lubiński

keywords

kidney kidney tumours nephron-sparing surgery

summary

Objective. Von Hippel-Lindau disease is characterized by an inherited
predisposition for tumour development. The most common manifestations
in this syndrome are haemangioblastomas of the central nervous system,
pancreatic cysts, pheochromocytomas, renal cysts and carcinomas. Almost
100% of solid renal tumours are renal cell carcinomas, very often bilateral
and multifocal. It is proved that distant metastases of renal cancer in VHL
patients with the primary lesion smaller than 3-5 cm in diameter do not occur.
It justifies the decision of watchful waiting or renal sparing procedures.
Materials and methods. We present our experience with 3 patients treated
at our clinic for renal cell carcinoma in association with VHL diseas. In all
cases nephron sparing surgery was performed. Oncological and functional
results of treatment are good.
Conclusions. Thorough follow-up of patients with VHL allows to initiate
proper surgical treatment in an ade±uate moment. Local tumour excision is
justified considering its common low grade, multifocal and recurrent character
and good treatment outcomes.

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