Abstract
Treatment of renal angiomyolipoma (AML) seeks to reduce related complications and preserve kidney function. The purpose of this article was to perform an updated literature review on the diagnosis, therapeutic options, and criteria for invasive intervention in patients with renal AML. Computerized tomography is the standard diagnostic method for renal AML, while definitive diagnosis is made by histopathology. The management of choice in most cases is active surveillance (AS), with a clinical and imaging follow-up protocol. In high-risk cases, therapeutic management should be considered, with alternatives such as selective arterial embolization (SAE), nephron-sparing surgery (NSS), and mTOR inhibitors in selected patients. Renal AML in women of childbearing age, those with growth >0.25 cm/year, intralesional aneurysms >5 mm, and clinically significant symptoms may qualify for active treatment. Despite the limitations derived from the available evidence, it is possible to consider SAE, NSS, and the use of mTOR inhibitors as management alternatives for selected patients.
Highlights
Renal angiomyolipoma (AML) is a rare benign renal tumor that is part of the group of perivascular epithelioid cell neoplasms (PEComas) (1, 2)
Fifty to seventy percent of cases correspond to sporadic renal AML, characterized by a smaller size, slow growth (0.19 cm/year), unilateral presentation, and an average age between 43 and 53 years at diagnosis (3, 5–7)
It should be kept in mind that this finding can be seen in renal cell carcinoma (RCC), lipoma, liposarcoma, oncocytoma, Wilms tumor, and teratoma; other characteristics such as the contrast enhancement pattern and the presence of calcifications must be considered (3, 4, 21)
Summary
Renal angiomyolipoma (AML) is a rare benign renal tumor that is part of the group of perivascular epithelioid cell neoplasms (PEComas) (1, 2). It is derived from mesenchyme, and is composed of mature adipose tissue, smooth muscle, and dysmorphic blood vessels (3). The remaining 30–50% is associated with genetic syndromes such as sporadic lymphangioleiomyomatosis (LAM) and tuberous sclerosis complex (TSC) (3, 4) The latter is due to an autosomal dominant mutation of the TSC1 (9q34) or TSC2 (16q13.3) genes, with activation of the mTOR intracellular signaling pathway, associated with a multisystemic disease, a greater number of lesions, a higher growth rate (1.25 cm/year), lower mean age at diagnosis (18 years), and considerable complications during follow-up (4, 8–10)
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