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Renal angiomyolipoma in Bourneville-Pringle syndrome
Article published in Urologia Polska 2005/58/1.

authors

Bartosz Małkiewicz, Janusz Dembowski, Michał Wróbel, Romuald Zdrojowy, Anna Kołodziej, Jerzy Lorenz
Katedra i Klinika Urologii i Onkologii Urologicznej Akademii Medycznej we Wrocławiu
Kierownik kliniki: prof. dr hab. Jerzy Lorenz

keywords

kidney, angiomyolipoma, Bourneville-Pringle syndrom

summary

introduction
Bourneville-Pringle syndrome is a rare genetically determined pathologic syndrome, observed at a 1/6000 - 1/14,500 incidence rate. The primary features of clinical presentation include: mental retardation, epilepsy and hard nodules located in the region of cheeks and nasobuccal folds. Ca 80% of patients with Bourneville-Pringle syndrome develop kidney lesions of angiomyolipoma (AML) type.
aim of the study
The paper discusses the Bourneville-Pringle syndrome, its etiology, clinical symptoms, diagnostics and treatment of angiomyolipomas (AML) coincident with TSC.
material and methods
Two cases of female patients with Bourneville-Pringle syndrome (TSC) with giant bilateral multifocal renal angiomyolipomas (AML) filling the whole abdominal cavity are presented. The patients underwent surgical treatment sparing the renal parenchyma. Improvement of the patients' clinical condition and normalization of renal function parameters, obtained during the post-operative period, were also observed in long-term follow-up.
conclusions
Multifocal character, considerable dynamics of growth, bilateral location, and, first of all, the tendency to reach big sizes typical of AML in patients with ST syndrome necessitates early and close radiological monitoring, and in each case requiring surgical treatment - consideration of surgery sparing the renal parenchyma in order to preserve normal renal function.

references

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correspondence

Bartosz Małkiewicz
Klinika Urologii i Onkologii Urologicznej AM
pl. 1 Maja 8
50-043 Wrocław
tel. (0... 71) 34 34 75
bmalkiew@urol.am.wroc.pl