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.
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