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Quiz Prize Rules Current Rankings DIAGNOSIS:
Bilateral Angiomyolipomas: Tuberous SclerosisBoth kidneys show increased parenchymal echogenicity with distorted parenchyma and echogenic lesions within. Echogenic mass lesions measuring approximately 15 cms in size are seen arising from the lower poles of both kidneys.
The most common cause of multiple echogenic lesions such as these in the kidneys is angiomyolipomas (AMLs) and the commonest cause of multiple AMLS is tuberous sclerosis.
AML or renal hamartoma is an uncommon benign tumour composed of varying degrees of smooth muscle cells and fat cells intermixed with thick-walled blood vessels.
AMLs may occur sporadically or in association with tuberous sclerosis. They are common in women with an F:M ratio of 2.3:1 to 25:2 with 80% occurring on the right side. Upto 50% cases of AML will have stigmata of tuberous sclerosis (mental retardation, epilepsy, sebaceous adenomas of face). Sixty-eighty % of tuberous sclerosis patients have AMLs, which are usually bilateral and multiple.
Most are asymptomatic and present when large. They may present with hematuria, flank pain or as flank masses.
On USG, they present as hypoechoic masses due to the high fat content with multiple non-fat interfaces, heterogeneous cellular architecture and/or numerous blood vessels. The more dense areas contain myomatous or vascular elements. Less echogenic areas are due to dilated calyces or areas of hemorrhage and necrosis. On very rare occasions, they are known to involve adjacent lymph nodes or the IVC. Due to lack of elastic tissue in blood vessels they are prone to form pseudoaneurysms.
The differential diagnosis for a small AML could be a renal cell carcinoma (RCC). However RCC generally is hypoechoic.
Small AMLs can be followed up for growth. For larger ones, renal sparing surgery can be performed. Embolization may be used for active bleeding.
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PubMed References for Bilateral Angiomyolipomas: Tuberous Sclerosis
At any given time this link will provide references from Pubmed, including the most recent updates. Retrievals are designed to include articles available free and also Indian studies.
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