Imaging of Renal Angiomyolipomatosis

Federico Greco,Vincenzo Cirimele,Bruno Beomonte Zobel,Pasquale D'Alessio,Carlo Augusto Mallio,Rosario Francesco Grasso

doi:10.15586/jrenhep.2018.37

Federico Greco, Vincenzo Cirimele + Show 4 more

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https://doi.org/10.15586/jrenhep.2018.37

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Abstract

Angiomyolipoma is a type of benign renal tumor. It is sporadic and isolated in 80% of cases. The remaining 20% is associated with tuberous sclerosis complex or pulmonary lymphangioleiomyomatosis. Generally, angiomyolipomas manifest themselves as angiomyolipomatosis, in which the angiomyolipomas are larger, bilateral, and widespread. Understanding whether angiomyolipomas are present in the context of angiomyolipomatosis is of considerable importance because it might be associated with malignant lesions. This article provides an overview of the radiological features of renal angiomyolipomatosis under different imaging techniques such as ultrasound, computed tomography, and magnetic resonance.

Highlights

Angiomyolipoma (AML) is a type of benign renal tumor, with an estimated prevalence of 0.3–3% of all renal tumors and a greater female predilection [1, 2]. It is characteristically a solid “triphasic” tumor composed of dysmorphic blood vessels, smooth muscle components, and mature adipose tissue which may be present in varying amounts [3]
Renal angiomyolipomatosis is a common manifestation in patients with tuberous sclerosis (TS) and LAM, where AMLs are larger, multiple, almost always bilateral, and have a greater predisposition to bleeding
AML is sporadic and isolated in 80% of cases, while the remaining 20% is associated with tuberous sclerosis complex (TSC) or pulmonary LAM [9, 10]

Summary

Introduction

Angiomyolipoma (AML) is a type of benign renal tumor, with an estimated prevalence of 0.3–3% of all renal tumors and a greater female predilection [1, 2]. Renal angiomyolipomatosis is a common manifestation in patients with tuberous sclerosis (TS) and LAM, where AMLs are larger, multiple, almost always bilateral, and have a greater predisposition to bleeding. As patients with TSC risk premature loss of nephrons due to increasing numbers and dimensions of cysts and AMLs, selective arterial embolization, percutaneous ablation, or partial nephrectomy are preferred conservative therapies for the treatment of these lesions [29]. These patients present a high risk of spontaneous hemorrhage; AML >4 cm and AML aneurysms >0.5 cm are risk factors for AML.

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