Primary hepatic epithelioid angiomyolipoma: a small case series.

Abstract

Hepatic epithelioid angiomyolipomas (HEAMLs) are rare and usually arise in case reports, and thus, we aimed to study the clinicopathological features, treatment and prognosis of these lesions. We identified patients from institutional database with HEAMLs and retrospectively collected clinical data. Of 12 patients, 10 were females, and the median age was 49 years. A liver mass was found on chance upon routine health screening in nine patients. Imaging features included clear border (9/12), internal heterogeneity (8/12), arterial enhancement (12/12), fat (5/12), intra-tumour vessel (3/12), draining hepatic vein (2/12) and pseudocapsule (1/12). The preoperative diagnoses contained HEAML (n=5), hepatocellular carcinoma (n=6), and hepatic cystadenocarcinoma (n=1). All cases had received surgery and obtained a negative margin. All specimens showed positive findings for HMB-45 and Melan A. No tumour recurrence or mortality was described with a mean follow-up time of 23.5months. HEAML is a low potential malignancy tumour that frequently appears in middle-aged females. It has nonspecific symptoms and may present special imaging features including intra-tumour vessel, early draining hepatic vein and lack of a pseudo capsule. The lesion is confirmed by pathological and immunohistochemical findings. Surgery and subsequent long-term follow-up are the most appropriate management approaches.