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

• 作者: Adam S Feldman Elahna Paul Elizabeth A Thiele Rahul A Sheth T Gregory Walker
• 来源期刊: 世界放射学杂志：英文版（电子版）
• 学科: 医学
• 关键词: Tuberous SCLEROSIS ANGIOMYOLIPOMA SIROLIMUS Angiography TRANSCATHETER embolization INTERVENTIONAL radiology Mammalian target of rapamycin inhibition EVEROLIMUS Volumetrics
• 年，卷（期）: 2016,（3）
• 页码范围: 308-315
• 页数: 8页
• 分类号: R593.2

摘要

AIM: To investigate the angiographic and volumetric effects of mammalian target of rapamycin(m TOR) 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 m TOR 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 toolwithin the ROI on post-contrast subtraction images. On computed tomography images, volume histogram analysis of Hounsfield unit was performed to quantify tumor tissue composition. The angiography procedures were also reviewed, and tumor vascularity based on pre-embolization angiography was characterized in a semi-quantitative manner. RESULTS: Patient 1 presented at the age of 15 with a 6.8 cm right lower pole AML and a 4.0 cm right upper pole AML. Embolization was performed of both tumors, and after a few years of size control, the tumors began to grow, and the patient was initiated on m TOR inhibitor therapy. There was an immediate reduction in the size of both lesions. The patient then underwent repeat embolization and discontinuation of m TOR inhibition, after which point there was a substantial regrowth in both tumors across all tissue compartments. Patient 2 presented at the age of 18 with a right renal AML. Following a brief period of tumor reduction after embolization, she was initiated on m TOR inhibitor therapy, with successful reduction in tumor size across all tissue compartments. As with patient 1, however, there was immediate rebound growth following disco