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NEW TRENDS IN RENAL ANGIOMYOLIPOMAS THERAPY


Authors: Tomáš Ürge 1;  Tomáš Pitra 1;  Zdeněk Chudáček 2;  Jiří Baxa 3;  Petr Stránský 1;  Viktor Eret 1;  Ondřej Hes 4;  Milan Hora 1
Authors place of work: Urologická klinika, Fakultní nemocnice Plzeň a LF UK v Plzni 1;  Radiodiagnostické oddělení, Fakultní nemocnice Plzeň a LF UK v Plzni 2;  Klinika zobrazovacích metod, Fakultní nemocnice Plzeň a LF UK v Plzni 3;  Šiklův ústav patologie, Fakultní nemocnice Plzeň a LF UK v Plzni 4
Published in the journal: Ces Urol 2015; 19(2): 106-117
Category: Review article

Summary

Renal angiomyolipoma (AML) is a benign mesenchymal tumor, which occurs sporadically or is associated with tuberous sclerosis complex (TSC). The overall prevalence of AML is 0.44 %, its incidence in women is 4 times higher than in men. Prevalence in the female population is thus 0.6 %, but male rates 0.28 %. Diagnosis is based on the presence of fat in the tumor. In most cases AML can be identified preoperatively. AML morphology is highly variable and in cases where fat is missing, preoperative diagnostic based on imaging methods is not possible. Active monitoring of patients with AML is crucial, because AML grows slowly and with minimal morbidity. Patient can present with haematuria, pain or retroperitoneal bleeding by tumors rupture. The main risk factors for bleeding are tumors volume, percentage of the vascular component in the tumor and the presence of TSC. Selective arterial embolization resolves hemorrhagic complications and reduces the volume of the tumor. The long-term outcomes of embolisation are poor due to tumor revascularization and growth. Nephron sparring surgery is associated with better long term outcomes. Radiofrequency ablation is another option, but strongly restricted by the volume tumor. An additional option is cryoablation, which has better results in cases where tumor is large, but this requires special equipment. Systematic treatment using mTOR inhibitors (everolimus, sirolimus), has recently been introduced, which are essential in patients with TSC, where the disease is often complicated by multiple and recurrent lesion. This therapy can help reduce the total volume of AML and allow eventual resection.

Key words:
Angiomyolipoma, arterial embolization, radiofrequeci ablation, cryoablation, resection, mTOR inhibitors.


Zdroje

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Štítky
Paediatric urologist Nephrology Urology
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