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Joseph Junewick, MD FACR
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Hepatic Angiomyolipoma

Case Detail

Anatomy: Gastrointestinal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Benign
Created: over 5 years ago
Updated: over 5 years ago
Tags: PEDS
Modality/Study Types: US
Activities:
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History

12 year old with tuberous sclerosis and hypertension.


Case Images


Diagnosis

Hepatic Angiomyolipoma

Findings

US – Multiple hyperechoic lesions of varying sizes in the kidneys and liver.

Discussion

Angiomyolipomas are benign mesenchymal neoplasms composed of an admixture of blood vessels, smooth muscle, and variable amounts of adipose tissue. Angiomyolipomas are associated with with the genes TSC1 and TSC2 that may act as tumor suppressor genes. Renal angiomyolipomas, occur in up to 80% of patients with tuberous sclerosis and larger ones are prone to spontaneous hemorrhage.

On ultrasound, these lesions are well defined rounded homogeneous hyperechoic masses easily separated from the normal hepatic parenchyma. Unenhanced CT shows these lesions as well-defined hypoattenuating lesions. whereas on contrast-enhanced CT the attenuation is typically heterogeneous because of the variable composition of the lesions.

Hepatic angiomyolipomas are more common in tuberous sclerosis patients with diffuse renal involvement. Additionally, females with tuberous sclerosis are more likely to have hepatic involvement. Hepatic lesions in a patient with tuberous sclerosis is not a cause for concern. Lesions larger than 4 cm in diameter may benefit from surveillance.

Reference

Fricke BL, Donnelly LF, Casper KA, et al. Frequency and Imaging Appearance of Hepatic Angiomyolipomas in Pediatric and Adult Patients with Tuberous Sclerosis AJR (2004); 182 (4): 1027-1030.



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