Percutaneous cryoablation for tuberous sclerosis-associated renal angiomyolipoma with neoadjuvant mTOR inhibition

BMC Urol. 2014 Sep 25:14:77. doi: 10.1186/1471-2490-14-77.

Abstract

Background: Renal angiomyolipomas (AMLs) are frequent in tuberous sclerosis and are responsible for a significant proportion of the morbidity in adulthood, mainly from bleeding complications, which are correlated to the size of the AMLs. We describe the case of a 19-year-old female with multiple bilateral renal angiomyolipomas.

Case presentation: The renal AMLs measured up to 6 cm in size. She was first treated with a low dose of the mammalian target of rapamycin (mTOR) inhibitor sirolimus (up to 3 mg/day over a 12-month period) and following significant AML size reduction, percutaneous cryoablation was performed. No side-effects of either treatment were reported. At 12 months post-cryoablation, no recurrence of the AML was noted.

Conclusion: This is the first report of this treatment strategy and the case study reveals that combining a low dose of an mTOR inhibitor with percutaneous cryoablation to treat small tumors mitigates the side-effects while providing a good clinical outcome. This therapeutic approach is a novel tool for the clinician involved in the management of patients with tuberous sclerosis.

MeSH terms

  • Adult
  • Angiomyolipoma / complications
  • Angiomyolipoma / pathology
  • Angiomyolipoma / therapy*
  • Cryosurgery / methods*
  • Female
  • Humans
  • Kidney Neoplasms / complications
  • Kidney Neoplasms / pathology
  • Kidney Neoplasms / therapy*
  • Neoadjuvant Therapy
  • Sirolimus / therapeutic use*
  • TOR Serine-Threonine Kinases / antagonists & inhibitors*
  • Treatment Outcome
  • Tuberous Sclerosis / complications*
  • Young Adult

Substances

  • TOR Serine-Threonine Kinases
  • Sirolimus