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Primary hepatic epithelioid angiomyolipoma: a small case series
Jisheng Zhu1, Guiyan Wang2, Gen Sun1
1Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China.
Background:
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.
Methods:
We identified patients from institutional database with HEAMLs and retrospectively collected clinical data.
Results:
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 23.5 months.
Conclusions:
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.
