Angiomyolipomas of the liver in tuberous sclerosis

Abstract

Although the association of tuberous sclerosis and renal angiomyolipomas is well established, the occurrence of hepatic hamartomatous lesions in this disease is less well documented. We describe two cases of tuberous sclerosis with associated multiple intrahepatic angiomyolipomas, and we review the literature on this subject. Radiologically, angiomyolipomas can range from entirely lipomatous to completely solid, features which are present in our cases. A characteristic finding of the hepatic involvement in this disease appears to be the multiplicity of the lesions. We suggest that in the setting of tuberous sclerosis it is reasonable to assume that intrahepatic lesions represent angiomyolipomas. However, in nontuberous sclerosis patients with similar findings malignancy cannot be excluded.

Coincidence of hepatocelluar carcinoma and hepatic angiomyolipomas in tuberous sclerosis complex: A case report

“…In patients with TSC, the incidence of renal angiomyolipoma has been reported in up to 80%, but hepatic angiomyolipoma has been less reported [3,4] . We recently encountered a patient with pathologic proved coincident hepatocellular carcinoma and hepatic angiomyolipomas in TSC.…”

Hepatic angiomyolipoma: Is fat in the liver friend or foe?

“…Interestingly enough, despite the enormous size of some of the AMLs described in this issue of Abdominal Imaging, no rupture has ever been recorded of a hepatic AML. In one of the cases, a patient with tuberous scle- rosis died of massive hemorrhage due to the rupture of a renal AML, but not from one in the liver [5].…”

“…In general, the diagnosis of tuberous sclerosis is known clinically, fat is detectable in all AMLs either small or large, there are multiple tumors, and there are associated renal AMLs [5,6].…”

Imaging findings of myomatous-type angiomyolipoma of the liver

“…The incidence of renal AML in cases of tuberous sclerosis has been reported to be 40%-80% (6), while only 5%-10% of patients with tuberous sclerosis have an AML of the liver (7). In most cases, hepatic AML occurs spontaneously, is not associated with renal locations (8), and is usually identified in adult women where its size may range from microscopic to giant. Most cases are asymptomatic and discovered incidentally, but they can be symptomatic and may occasionally rupture (9).…”