Angiomyolipoma (AML) arising in the liver is rare and usually benign, but it occasionally has malignant potential. A 58-year-old man with a liver tumor identified by a previous doctor with features suggestive of hepatocellular carcinoma on computed tomography (CT) underwent anterior segmentectomy of the liver in 2006. Microscopically, the tumor was composed of exclusively epithelioid cells that were scatteredly positive for human melanoma black 45 on immunohistochemistry. Accordingly, primary hepatic epithelioid AML (eAML) was diagnosed. The patient was subsequently referred to our hospital for follow-up after hepatectomy. He had event-free survival for nearly 7 years. In 2013, two well-defined round nodules were detected in the right lung field by chest CT, and partial pneumonectomy was performed for diagnosis and treatment. Histological examination of the resected lung tissue showed that it was morphologically and immunohistochemically identical to his primary hepatic eAML, leading to the diagnosis of pulmonary metastasis. This paper demonstrates a rare case of malignant hepatic eAML with late recurrence in the lung after hepatectomy.
Keywords: Hepatic angiomyolipoma; Malignant potential; Perivascular epithelioid cell.