Nephron-sparing resection of angiomyolipoma after sirolimus pretreatment in patients with tuberous sclerosis.
OBJECTIVE
Renal angiomyolipoma in patients with tuberous sclerosis can cause life-threatening bleeding. Embolization or resection is recommended, but either intervention may result in substantial loss of renal function. Recently, regression of angiomyolipoma size has also been achieved with mTOR inhibitor therapy, but recurrent lesion growth after treatment cessation has to be expected. This is the first report on a multimodal therapeutic approach facilitating a nephron-sparing, definitive resection.


METHODS
Three patients with renal angiomyolipoma not amenable to nephron-sparing surgery were treated with sirolimus aiming trough levels of 4-8 ng/ml. Treatment was well tolerated.


RESULTS
mTOR inhibitor treatment resulted in a regression of angiomyolipoma volume by 38-95 %. Thereafter, nephron-sparing angiomyolipoma resection conserved normal renal function.


CONCLUSIONS
Neoadjuvant use of mTOR inhibitor pretreatment may represent a novel approach facilitating nephron-sparing resection.
Urologische Klinik, Klinikum der Ludwig-Maximilians Universitaet, Marchioninistr. 15, 81337, Munich, Germany.
Michael
M
Staehler
Matthias
M
Sauter
Andreas
A
Helck
Ulrich
U
Linsenmaier
Lutz
L
Weber
Karin
K
Mayer
Michael
M
Fischereder
D018207:Angiomyolipoma / Q000601:surgery*; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use*; D002648:Child; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications* / Q000601:surgery*; D008297:Male; D009392:Nephrectomy / Q000379:methods*; D009399:Nephrons; D020123:Sirolimus / Q000627:therapeutic use*; D014402:Tuberous Sclerosis / Q000150:complications*; D055815:Young Adult
D000903:Antibiotics, Antineoplastic; D020123:Sirolimus