Treatment modalities for renal angiomyolipomas associated with tuberous sclerosis

DOI: 10.22591/magyurol.2017.2.szendroia.69

Authors:
Szendrői Attila dr. (Semmelweis Egyetem, Urológiai Klinika, Budapest (igazgató: Nyirády Péter dr.))

Summary

Sclerosis Tuberosa (TSC) is an autosomal dominantly inherited, various organs such as kidney in 80%, brain in 75%, skin in 70%, and heart in 50% affecting disease. In the patient’s kidney next to multiple cysts angiomyolipomas (AML) also appear, both tend to ruin the renal function on long-term. In case of sclerosis tuberose the clinical appearance of the angiomyolipomas differ from the sporadic AML: they appear in younger age, show progressive growing, with several centre and both kidneys are involved. Treatment of AML in urology is performed due to the high risk of heavy bleeding or blood shock and means in most of the cases kidney resection or nephrectomy. On the other hand surgery is not ideal on TS based AML, because we have to count on further manifestations of the illness and repeated surgeries cause damage to the renal parenchyma and even without surgery complications this increases the development of renal insufficiency. This enhances the importance of the minimal invasive radiological interventions, such as the selective embolization and radiofrequency ablation the importance of the medical treatment. Per os applying the mTOR inhibitor everolimus on regular basis, the size of the AML’s decrease, preventing the shock of bleeding. On the systematic therapy the other organ’s lesions react also well, there will be less epileptic attacks; cognitive functions will be better, etc. The treatment of TS patients is a multidisciplinary task, where the patient’s quality of life and the expected lifetime can be supported by the efficient cooperation of the different professions.

click here to read the full article