Pathological and molecular analysis of sporadic hepatic angiomyolipoma

Hepatic angiomyolipoma (HAML) is an unusual mesenchymal lesion that can be misdiagnosed as hepatocellular carcinoma or sarcoma. We sought to better define the morphological variations and immunohistochemical and molecular features of this unusual tumor. Forty-nine sporadic HAMLs were investigated for immunopathologic characteristics with EnVision Plus. The clonal analysis was based on the methylation pattern of the polymorphic X chromosome-linked human androgen receptor gene, loss of heterozygosity (LOH), and microsatellite instability (MSI) detected with polymerase chain reaction using the MegaBACE 500 automatic system on microdissected tissues. Histologically, HAML is composed of a heterogeneous mixture of blood vessels, smooth muscle, and adipose cells. The myomatous or myoid cells were the most variable. Most of the tumor cells were positive for HMB-45 (100%) and SMA (100%). There was a typical monoclonal pattern in 35 of the 40 tumors. No LOH or MSI was found. Hepatic AML is a benign neoplasm with varied morphology and monoclonal growth. HMB-45 is the best marker available for diagnosis. Neither LOH nor MSI appears to play an important role in the pathogenesis of this tumor.