doiSerbia

Bilateral angiomyolipoma of the kidney in patient with tuberous sclerosis

Čolović Radoje (Institut za bolesti digestivnog sistema, Klinički centar Srbije, Beograd)
Čolović Nataša (Institut za hematologiju, Klinički centar Srbije, Beograd)
Grubor Nikica (Institut za bolesti digestivnog sistema, Klinički centar Srbije, Beograd)
Radak Vladimir (Institut za bolesti digestivnog sistema, Klinički centar Srbije, Beograd)
Micev Marijan (Institut za bolesti digestivnog sistema, Klinički centar Srbije, Beograd)
Stojković Mirjana (Institut za bolesti digestivnog sistema, Klinički centar Srbije, Beograd)

Angiomyolipomas are relatively frequent tumors of the kidney. It is believed that about 10 million people worldwide have such a tumor. About 1/10 of these 10 million are patients who suffer from tuberous sclerosis. The tumors are frequently bilateral, slow growing, and usually a symptomatic, as well as being rare in children. Due to the benign nature of angiomyolipomas, surgical treatment and embolisation of the tumors are generally not recommended, unless renal function is endangered, the symptoms are severe, or the kidney in question becomes completely dysfunctional. This is particularly the case in patients with tuberous sclerosis in whom these tumors are either already bilateral or may become so. We present a 24-year-old woman with tuberous sclerosis in whom bilateral kidney tumors were diagnosed 7 years earlier and in whom we carried out a left nephrectomy of a 5300 gram angiomyolipoma, which caused pain and complete loss of function. Although timorous, the right kidney was functional, so it was left untouched. After an uneventful recovery, a close follow-up was recommended, as well as HLA typing, as it is highly probable that the right kidney will gradually become inadequate or completely dysfunctional, so that haemodialysis and/or kidney transplantation along with nephrectomy will become necessary.

Keywords: tuberous sclerosis, kidney, angiomyolipoma