Hepatic angiomyolipoma (AML) is very rare and only about 80 cases have
been reported. The tumor is fundamentally heterogeneously composed of
the three tissue components of blood vessels, smooth muscle cells (SM
C), and fat cells. Two cases of hepatic AML are reported here, both of
which are histologically composed predominantly of a fat cell element
and resembled true lipoma (lipomatous AML). However, careful examinat
ion of both tumors revealed the presence of a small amount of epitheli
oid SMC, especially around blood vessels. Immunohistochemical study us
ing monoclonal antibody for melanoma (HMB-45) clearly revealed a small
amount of HMB-45-positive SMC around the blood vessels and scattered
in the diffuse fat cell growth in both tumors. Since no liver tissue c
omponents or primary liver tumors are reactive with HMB-45 except AML
cells, the presence of HMB-45-positive cells within the tumor clearly
established the diagnosis of hepatic AML. Any fatty tumor or focal fat
ty lesion of the liver that superficially resemble true lipomas should
be tested for the presence of HMB-45-positive SMC in the tumor to dif
ferentiate it from AML.