Re: Everolimus Therapy for Angiomyolipoma in Patients with Tuberous Sclerosis Complex or Sporadic Lymphangio- leiomyomatosis: Results from EXIST-2

The past year has seen two important milestones in the experience with everolimus reveals uniform responses on the various manifestations of TSC, including the central nervous system (subependymal giant cell astrocytomas), skin lesions, and renal AML. Although surgical management remains the standard of care and the only form of therapy capable of consistently achieving a ‘‘complete remission’’ for renal cell carcinoma and symptomatic or large AML, the addition of these targeted agents adds an importantmedical element, allowing a new paradigm for multimodality management, such as when renal preservation may be difficult without some form of preinterventional cytore-duction. There is also some evidence that the rare and highlymalignant epithelioid variant of AMLmay respond to everolimus [2], and newer-generation agents with poten