Malignant potential of epithelioid angiomyolipomas of the liver: A case report and comprehensive review of the literature.

Hepatic epithelioid angiomyolipoma (HEAML) is a rare mesenchymal tumor that has been reported to have malignant potential. We herein describe a rare case of atypical HEAML. A 43-year-old Caucasian male patient visited his general practitioner due to a productive cough persisting for >2 months. During evaluation with several imaging tests, a chest computed tomography (CT) incidentally revealed a well-demarcated lesion in the caudate lobe of the liver, sized 7 cm. An abdominal magnetic resonance imaging examination confirmed the findings of the CT. The possibility of hepatocellular carcinoma at that time could not be excluded. Due to inconclusive cross-sectional imaging, the patient underwent left hepatectomy with additional resection of segment I. The patient's postoperative course was uneventful. A diagnosis of 'atypical' HEAML was established in the present case. The majority of HEAMLs are considered to be benign, although there are several reported cases exhibiting malignant behavior, such as tumor growth, presence of atypical cells, recurrence after surgical resection, metastasis and invasive growth into the liver parenchyma and alongside the vessels. From 2000 onwards, 19 cases of malignant hepatic AML have been reported. Malignant transformation is considered to occur mostly in the epithelioid subtype. To that end, when epithelioid or atypical characteristics are identified on preoperative biopsy, resection is indicated due to the high probability of malignancy.