A clinical study on 17 patients with liver perivascular epithelioid cell tumors / 中华肝胆外科杂志

ABSTRACT

Objective:To study the clinical manifestations and prognosis of patients with liver perivascular epithelioid cell tumor (PEComa).Methods:The clinical manifestations, imaging findings, pathological features, treatment and prognosis of 17 patients with liver PEComa managed in Shenzhen People's Hospital from September 2002 to January 2020 were retrospectively analysed.Results:The patient age of onset of liver PEComa was 22 to 54 years (average 34.8 years). There were 13 females and 4 males. 82%(14/17) of patients presented with no significant symptoms. 15/17 had solitary tumors. The diagnostic rate using preoperative B-ultrasound, CT and MRI was low. Postoperative pathological studies showed the tumor cells were mainly epithelioid cells with hyaline or eosinophilic cytoplasm. There were abundant blood vessels in the tumor tissues. Adipose tissues were also seen. Immunohistochemistry showed both HMB45 and Melan-A positivities to arrive at the diagnosis. All patients were treated with surgical resection. There were no recurrence, metastasis, or death on follow-up.Conclusion:Hepatic PEComa occurred more frequently in young women and it had no specific clinical manifestations. Except for a small number of tumors with adipose tissue detected on preoperative imagings, this tumor was difficult to diagnose, or even be suspected on preoperative imagings. Pathological examination combined with immunohistochemistry after surgery established the diagnosis. Surgery resulted in good prognosis.