Hepatic epithelioid angiomyolipoma with an unusual pathologic appearance: expanding the morphologic spectrum.
نویسندگان
چکیده
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.
منابع مشابه
Perivascular Epithelioid Cell Tumor: A Rare Liver Primary of Mesenchymal Origin
Perivascular Epithelioid Cell Tumors (PEComas) are a family of rare neoplasms defined as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells [1]. Pea et al. first noted this unusual cell type in both renal angiomyolipoma (AML) and clear-cell “sugar” tumor (CCST) of the lung [2]. This generated the concept of a family of neoplasms co...
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BACKGROUND Hepatic angiomyolipomas are a rare, benign group of mesenchymal tumors in the liver. Hepatic angiomyolipoma is sometimes misdiagnosed as hepatocellular carcinoma, and there is the possibility of a malignant transformation. Hence, the accurate diagnosis of this disorder is necessary. CASE PRESENTATION A 64-year-old Japanese man was observed to have a space-occupying lesion of 15-mm ...
متن کاملColonic angiomyolipoma with a monotypic expression and a predominant epithelioid component.
Angiomyolipomas are rare lesions, often arising in the kidney, and are part of a group of tumours with a diverse appearance and evidence of dual melanocytic and smooth muscle differentiation known as PEComas (tumours of perivascular epithelioid cell origin). This report describes an unusual case of a colonic PEComa in a 40 year old woman. Unlike most of the previous colonic angiomyolipomas/PECo...
متن کاملCASE REPORT Colonic angiomyolipoma with a monotypic expression and a predominant epithelioid component
Angiomyolipomas are rare lesions, often arising in the kidney, and are part of a group of tumours with a diverse appearance and evidence of dual melanocytic and smooth muscle differentiation known as PEComas (tumours of perivascular epithelioid cell origin). This report describes an unusual case of a colonic PEComa in a 40 year old woman. Unlike most of the previous colonic angiomyolipomas/ PEC...
متن کاملLaparoscopic Resection of Hepatic Angiomyolipoma – An Uncommon Primary Liver Tumor: A Case Report and Literature Review
PEComas are an uncommon group of mesenchymal neoplasms that exhibit perivascular epithelioid cell differentiation. The term PEComa includes a collection of different subcategories, such as lymphangioleimyomatosis, clear cell tumor of the lung, and angiomyolipoma (AML) which is the topic of discussion in this article. The main problem concerning the diagnosis of hepatic AML is the wide non-speci...
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ورودعنوان ژورنال:
- International journal of clinical and experimental pathology
دوره 7 9 شماره
صفحات -
تاریخ انتشار 2014