title: Hepatic epithelioid angiomyolipoma with an unusual pathologic appearance: expanding the morphologic spectrum.
abstract: Hepatic epithelioid angiomyolipoma (AML) is a rare lesion that is characteristically composed of a predominant or exclusive population of epithelioid cells coexpressing melanocytic and myogenic markers. The cystic variant of epithelioid AML is exceedingly uncommon. In this study, we present the clinicopathological features of a case of hepatic epithelioid AML with remarkable cystic degeneration in a 34-year-old female as well as with a literature review. A magnetic resonance imaging scan revealed a well-defined 30 cm × 25 cm hepatic mass. Sectioning of the well-defined mass revealed a non-encapsulated tumor that was multiloculated with amorphous necrotic tissue and hemorrhagic fluid. The inner cystic wall was rough and brownish-black in color. Microscopically, the tumor largely consisted of epithelioid cells that comprised approximately 95% of the total neoplastic components but also contained some spindle myoid cells, mature fat, and a thick-walled vasculature. Both intracellular and extracellular hyaline globules were frequently identified. Necrosis and invasive growth patterns were also present. By immunohistochemistry, spindle-epithelioid neoplastic cells were variably positive for Melan-A, HMB45, and SMA but were uniformly negative for epithelial and hepatocytic markers. This is the third report of a cystic AML in liver. The patient was followed for 71 months without any evidence of metastasis or recurrence.
authors.affiliation: Department of Liver Disease, The Military General Hospital of Beijing PLA Beijing 100700, China.
authors.forename: Yiming
authors.initials: Y
authors.lastname: Zhou
authors.affiliation: Department of Surgery, The Sixth Hospital of Jinan City Zhangqiu, Shandong Province, China.
authors.forename: Fangshu
authors.initials: F
authors.lastname: Chen
authors.affiliation: Department of Surgery, The Sixth Hospital of Jinan City Zhangqiu, Shandong Province, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Jiang
authors.affiliation: Department of Surgery, The Sixth Hospital of Jinan City Zhangqiu, Shandong Province, China.
authors.forename: Qingda
authors.initials: Q
authors.lastname: Meng
authors.affiliation: Department of Pathology, 309 Hospital of People's Liberation Army Beijing, China.
authors.forename: Fenghua
authors.initials: F
authors.lastname: Wang
mesh_terms: D000328:Adult; D018207:Angiomyolipoma / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D002763:Cholecystectomy; D015622:Epithelioid Cells / Q000737:chemistry / Q000473:pathology*; D005260:Female; D006498:Hepatectomy; D006801:Humans; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor
keywords: Angiomyolipoma; cystic degeneration; liver; perivascular epithelioid cell tumor