International Urology and Nephrology

, Volume 44, Issue 6, pp 1657–1661

Nephron-sparing resection of angiomyolipoma after sirolimus pretreatment in patients with tuberous sclerosis

  • Michael Staehler
  • Matthias Sauter
  • Andreas Helck
  • Ulrich Linsenmaier
  • Lutz Weber
  • Karin Mayer
  • Michael Fischereder
Urology - Original Paper

DOI: 10.1007/s11255-012-0292-z

Cite this article as:
Staehler, M., Sauter, M., Helck, A. et al. Int Urol Nephrol (2012) 44: 1657. doi:10.1007/s11255-012-0292-z

Abstract

Purpose

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.

Conclusion

Neoadjuvant use of mTOR inhibitor pretreatment may represent a novel approach facilitating nephron-sparing resection.

Keywords

Tuberous sclerosisAngiomyolipomamTOR inhibitorSirolimusNephron-sparing surgeryRenal function

Copyright information

© Springer Science+Business Media Dordrecht 2012

Authors and Affiliations

  • Michael Staehler
    • 1
    • 6
  • Matthias Sauter
    • 2
    • 6
    • 7
  • Andreas Helck
    • 3
  • Ulrich Linsenmaier
    • 3
    • 6
  • Lutz Weber
    • 4
    • 6
  • Karin Mayer
    • 5
    • 6
  • Michael Fischereder
    • 2
    • 6
  1. 1.Urologische KlinikKlinikum der Ludwig-Maximilians UniversitaetMunichGermany
  2. 2.Medizinische Klinik und Poliklinik IVKlinikum der Ludwig-Maximilians UniversitaetMunichGermany
  3. 3.Institut fuer Klinische Radiologie KlinikKlinikum der Ludwig-Maximilians UniversitaetMunichGermany
  4. 4.Dr. von Haunersche’s KinderspitalKlinikum der Ludwig-Maximilians UniversitaetMunichGermany
  5. 5.Zentrum fuer Humangenetik und LaboratoriumsmedizinMartinsriedGermany
  6. 6.Tuberöse Sklerose ZentrumKlinikum der Ludwig-Maximilians UniversitaetMunichGermany
  7. 7.Tuberöse Sklerose Zentrum - Medizinische Klinik und Poliklinik IVKlinikum der Ludwig-Maximilians UniversitaetMunichGermany