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. 2022 Apr 13;31(4):284-288.
doi: 10.1055/s-0042-1743253. eCollection 2022 Dec.

Complex Arterial Pathology of Angiomyolipomas of Tuberous Sclerosis

Christopher D Yeisley  1 , Shima Tafreshi  1 , Joseph Moirano  2 , Eric Gandras  1 , David Siegel  1
Affiliations

Complex Arterial Pathology of Angiomyolipomas of Tuberous Sclerosis

Christopher D Yeisley et al. Int J Angiol. .

Abstract

Based on observations at angiography, it was hypothesized that angiomyolipomas (AMLs) associated with tuberous sclerosis complex (TSC) have a more robust parasitic blood supply and require more complex embolization involving lower order vessels compared with the non-TSC patients' AMLs. This was a retrospective review of 71 patients who underwent angiography and embolization for renal AML at multiple centers within a single health system. All of the patients with TSC (11/11) were found to have "complex" tumor vascular supply. Of the patients with sporadic AML, 51/60 (85%) had "simple" tumor vascular supply. Sporadic cases with a complex tumor blood supply were larger tumors, with an average size of 11.5 cm. This study supports our hypothesis that AMLs associated with TSC have more complex tumor vascularity compared with sporadic lesions, and aims to better prepare interventionalists to deal with the complex cases.

Keywords: AML; complex tumor vascularity; embolization; hemorrhage; tuberous sclerosis; tuberous sclerosis complex; tumor.

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Conflict of interest statement

Conflict of Interest None declared.

Figures

Fig. 1
Fig. 1
A 51-year-old woman who presented for embolization of a sporadic renal AML that was increasing in size. ( A ) Axial CT without IV contrast demonstrating a left renal lesion containing bulk fat (arrow). ( B ) Axial in-phase sequence MR demonstrating a left renal lesion (arrow). ( C ) Axial opposed-phase sequence MR shows decreased signal of the left renal lesion (arrow) consistent with microscopic fat. ( D ) Arteriography of the left kidney with abnormal tumor vascularity arising from the left lower pole. The tumor is only supplied by a fourth-order vessel (arrow), consistent with simple tumor vascularity. AML, angiomyolipoma; CT, computed tomography; IV, intravenous; MR, magnetic resonance.
Fig. 2
Fig. 2
A 45-year-old woman who presented for embolization of a sporadic renal AML that spontaneously hemorrhaged. ( A ) Transverse sonographic image of the kidney showing a hyperechoic mass (arrows) that displaces the left kidney. ( B ) Coronal CT with intravenous contrast showing a large mass arising from the left kidney (arrow), which contains bulk fat and areas of abnormal enhancement. ( C ) Proximal arteriography demonstrates a large tumor arising from the left kidney with abnormal tumor vascularity (arrow). ( D ) Subselective arteriography was performed after embolization of one of the higher order feeding vessels (arrowhead), which demonstrated persistent tumor vascularity (arrow), consistent with complex tumor vascularity. ( E ) Completion arteriography demonstrates that the tumor, as well as a portion of normal tumor parenchyma, has been devascularized. AML, angiomyolipoma; CT, computed tomography.
Fig. 3
Fig. 3
A 43-year-old woman who presented with abdominal pain and was found to have a large AML with retroperitoneal hemorrhage. ( A ) Persistent abnormal tumor vascularity (arrow) after glue embolization (arrowhead) of a third-order arterial branch. ( B ) Multiple additional third-order vessels were embolized (arrowheads) to revascularize the tumor, consistent with complex tumor vascularity. AML, angiomyolipoma.
Fig. 4
Fig. 4
A 29-year-old with tuberous sclerosis presented for embolization of multiple AMLs increasing in size. ( A ) Proximal left renal angiogram demonstrates multiple areas of abnormal tumor vascularity in the upper and lower poles of the kidney. ( B ) Selective angiogram from a second-order upper pole renal artery demonstrates complex tumor vascularity to the AML. ( C ) Particle embolization was performed at the level of a third-order upper pole branch with diluted contrast demonstrating stasis (arrow). ( D ) Postembolization arteriography shows persistent tumor vascularity from third-order vessels (arrow). AML, angiomyolipoma.
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