Malignant hepatic perivascular epithelioid cell tumor: A case report and literature review.

Perivascular epithelioid cell tumors (PEComas) comprise a rare group of mesenchymal neoplasms, which can arise from almost any location in the body, but cases of primary hepatic PEComa are extremely rare. Here we report a hepatic PEComa in a 30-year-old man with abdominal pain . An extended left hepatectomy was proceeded and the results showed negative margins. Histologically, the tumor cells presented an epithelioid morphology with polymorphic nuclei and abundant eosinophilic cytoplasm and focal vascular infiltration. Necrotic tissue was present, closely resembling hepatocellular carcinoma. Immunohistochemistry demonstrated strong expression of HMB-45, CD34 and Ki67. Imaging findings and molecular analysis supported the malignant hepatic PEComa. Additionally, surgical resection is the first line treatment, and targeted therapy offers a viable therapeutic approach for individuals with inoperable malignant tumors and for those in the postoperative period.