Clin Mol Hepatol.  2017 Mar;23(1):80-86. 10.3350/cmh.2016.0034.

Hepatic perivascular epithelioid cell tumor (PEComa): a case report with a review of literatures

• Son HJ
• Kang DW
• Kim JH
• Han HY
• Lee MK

• KMID: 2412118
• DOI: http://doi.org/10.3350/cmh.2016.0034

Abstract

Hepatic perivascular epithelioid cell tumors (PEComas) are very rare. We report a primary hepatic PEComa with a review of the literature. A 56-year-old women presented with a nodular mass detected during the management of chronic renal failure and chronic hepatitis C. Diagnostic imaging studies suggested a nodular hepatocellular carcinoma in segment 5 of the liver. The patient underwent partial hepatectomy. A brown-colored expansile mass measuring 3.2×3.0 cm was relatively demarcated from the surrounding liver parenchyma. The tumor was mainly composed of epithelioid cells that were arranged in a trabecular growth pattern. Adipose tissue and thick-walled blood vessels were minimally identified. A small amount of extramedullary hematopoiesis was observed in the sinusoidal spaces between tumor cells. Tumor cells were diffusely immunoreactive for human melanoma black 45 (HMB45) and Melan A, focally immunoreactive for smooth muscle actin, but not for hepatocyte specific antigen (HSA).

Keyword

Hepatic; PEComa; HMB45; Melan A

MeSH Terms

Actins/metabolism
Antibodies, Viral/blood
Female
Hepatitis C, Chronic/complications
Humans
Liver Neoplasms/complications/*diagnosis/pathology
MART-1 Antigen/metabolism
Melanoma-Specific Antigens/metabolism
Microscopy, Fluorescence
Perivascular Epithelioid Cell Neoplasms/complications/*diagnosis/pathology
Tomography, X-Ray Computed
Ultrasonography
Actins
Antibodies
MART-1 Antigen
Melanoma-Specific Antigens