Angiographic and volumetric effects of mammalian target of rapamycin inhibitors on angiomyolipomas in tuberous sclerosis

Abstract

AIM:To investigate the angiographic and volumetric effects of mammalian target of rapamycin (mTOR) inhibitors on angiomyolipomas (AMLs) in a case series of patients with tuberous sclerosis complex. METHODS:All patients who underwent catheter angiography prior to and following mTOR inhibitor therapy (n = 3) were evaluated. All cross-sectional imaging studies were analyzed with three-dimensional volumetrics, and tumor volume curves for all three tissue compartments (soft tissue, vascular, and fat) were generated. Segmentation analysis tools were used to automatically create a region of interest (ROI) circumscribing the AML. On magnetic resonance images, the "fat only" map calculated from the in-and opposed-phase gradient recalled echo sequences was used to quantify fat volume within tumors. Tumor vascularity was measured by applying a thresholding tool

Outcomes of everolimus treatment for renal angiomyolipoma associated with tuberous sclerosis complex: A single institution experience in Japan

“…In the present study, the rate was 68% (32/47), showing a higher tumor size‐reducing effect than in the EXIST‐2 study. Everolimus is estimated to shrink the vascular and muscle components of AML . Thus, AML with highly vascular and muscle components might decrease rapidly.…”

“…Everolimus is estimated to shrink the vascular and muscle components of AML. 17 Thus, AML with highly vascular and muscle components might decrease rapidly. We completed treatment when everolimus reduced the AML size to 4 cm or smaller.…”

Treatment of Renal Angiomyolipoma and Other Hamartomas in Patients with Tuberous Sclerosis Complex

“…Unlike oncology care, where the goal of treatment is to rid the patient of every last vestige of tumor cells, in angiomyolipoma, small stable lesions are not a problem for long-term survival or symptom relief. Although there is evidence that halting treatment with an mTOR inhibitor will likely lead to regrowth of the angiomyolipomas (38,50), whether reduction in the dose of an mTOR inhibitor is possible after lesions have reached a small stable size is unclear.…”

Editorial Comment to Outcomes of everolimus treatment for renal angiomyolipoma associated with tuberous sclerosis complex: A single institution experience in Japan

“…Regrowth was found across all three components, fat, soft tissue and vascular components, after the discontinuation of everolimus. 4 Do all AML begin to regrow after everolimus discontinuation? Furthermore, is the re-administration of everolimus effective for regrown AML?…”

“…reported in a case series of three TSC patients with AML that AML immediately started to regrow to their original size or larger after everolimus was discontinued. Regrowth was found across all three components, fat, soft tissue and vascular components, after the discontinuation of everolimus . Do all AML begin to regrow after everolimus discontinuation?…”