Intractable Rare Dis Res. 2022;11(4):202-205. (DOI: 10.5582/irdr.2022.01111)

Insights into clinical diagnosis and treatment of malignant hepatic perivascular epithelioid cell tumor

Li Z, Zhou Y, Wang C, Yu H, Yang G, Ma Y


Perivascular epithelioid cell tumors (PEComas) are infrequent mesenchymal tumors. They are usually benign, and only a few are malignant. These tumors are more commonly found in middle-aged women. PEComas are mainly composed of differentiated perivascular epithelioid cells arranged radially around the vascular cavity, and they are usually positive for melanocyte markers and smooth muscle cell differentiation markers. Among the PEComas, hepatic PEComas generally have no obvious symptoms and no typical imaging manifestations. Malignant hepatic PEComas are even rarer. So, we explained our insights into clinical diagnosis and treatment of malignant hepatic PEComas, in order to help clinicians and pathologists to further understand PEComas.

KEYWORDS: PEComa, liver tumor, hepatectomy, mTOR inhibitors, TFE3

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