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EARLY DIAGNOSIS OF ASYMPTOMATIC RENAL SOLID TUMORS IN VHL FAMILIES IN POLAND
Article published in Urologia Polska 1998/51/2.

authors

Karol Krzystolik, Cezary Cybulski, Jan Lubiński, Stanisław Zaj±czek, Monika Sochańska, Aleksandra Tołoczko, Grażyna Psut, Monika Górecka, Grażyna Wilk, Włodzimierz Paprzycki, Andrzej Sikorski, Krzysztof Czernicki, Marcin Słojewski, Bartłomiej Gliniewicz, Zofia Krzystolik, Wojciech Lubiński, Ewa Starzycka-Bigaj, Marek Prost, Ewa Kostyk, Małgorzata Zdunek, Aleksandra Omulecka, Jan Jaskólski, Bronisława Koraszewska-Matuszewska, Daniel Chibowski, Renata Zalewska, Olga Haus, Katarzyna Zasada, Ireneusz Kojder, Robert Kaczor, Jan ¦lósarek, Elżbieta Włodarczyk, Zygmunt Domański, Grażyna Malukiewicz, Jerzy Bidziński, Józef Kałuża, Barbara Wysocka, Janusz Limon, Sergiusz JóĽwiak
O¶rodek Nowotworów Dziedzicznych przy Zakładzie Genetyki i Patomorfologii Pomorskiej AM i Regionalnego Szpitala Onkologicznego w Szczecinie
przy współpracy Centrum Zdrowia Dziecka i AM w Białymstoku, Bydgoszczy, Gdańsku, Katowicach, Krakowie, Lublinie, Łodzi, Poznaniu, Szczecinie, Warszawie i Wrocławiu
Koordynator programu: prof. dr hab. J. Lubiński

keywords

kidney renal cell carcinoma von Hippel-Lindau disease

summary

Objective. Von Hippel-Lindau disease (VHL) is one of hereditary cancer
predisposition syndromes. Classical lesions affect: 1) central nervous system
iCNS) ? haemangioblastomas, 2) retina ? haemangioblastomas, 3) pancreas
? cysts, island cell tumors and cancer, 4) epididymis ? cystadenoma papillare,
5) adrenal glands and paraganglia ? pheochromocytoma and paraganglioma,
t) endolymphatic sac tumors ELST and 7) renal cysts and cancer (RCC). Mean
age of death of VHL patients is about 41 years. As neurosurgical techni±ues
are becoming more advanced, RCC is becoming the most fre±uent cause of
death in these patients. The aim of the work was to evaluate the use of
screening program in VHL patients in early detection of asymptomatic renal
solid tumors. In Poland such screening programs in VHL patients have not
been widely used.
Patients and methods. Ultrasound scan (from age of 11) and CT (from age
of 20) was performed in 30 patients with definitive diagnosis of VHL. Control
group consisted of non-VHL patients, age and sex matched, in whom
ultrasound scan was performed because of other than urinary tract indications.
Results. Asymptomatic solid tumors of kidney were found in 12 of 30 VHL
patients (40%). Mean age of diagnosis was 38.6. Tumors were multifocal in 9
9/12), bilateral in 6 (6/12), and coexisting cysts in 11 (11/12
local or distant metastases were detected.
Conclusions. 1. Most of VHL patients in Poland were not properly screened
for renal lesions. 2. Starting the screening program for VHL patients enabled
to diagnose early asymptomatic solid tumors in 40% of the patients.

references

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