[Clinical analysis of hepatic perivascular epithelioid cell neoplasm: a report of eleven cases]

Objective: To investigate the clinical features, diagnosis and therapy of hepatic perivascular epithelioid neoplasms (PEComa). Methods: The clinical data of eleven patients with hepatic PEComa who received surgical treatment at Shengjing Hospital Affiliated to China Medical University from April 2012 to October 2017 were collected. The clinical manifestations, imaging features, diagnostic and therapeutic strategies, pathologic features, prognosis were analyzed. Results: The patients aged from 35 to 55 years (mean: 47 years , 3 males and 8 females). Two patients had epigastric pain, the others rarely had any clinical symptom. Hepatitis C virus (HCV) infection was present in one patient 9.09%(1/11), the rate of correct diagnosis by imageological examination before operation was only 9.09%(1/11). All patients received a surgical resection, the final diagnosis of hepatic PEComa was made with pathology and immunohistochemistry. The antibodies used for immunohistochemistry varied from patient to patient. The positive rates of Melan A, HMB45, smooth muscle actin and S-100 were 100%(10/10), 90%(9/10), 77.8%(7/9)and 33.3%(3/9) respectively, the Ki-67 positive index was 1%-10%. One patient died after surgery because of hemorrhage, other ten patients received long-term follow-up(5-71 months), and no recurrence or metastasis was observed. Conclusion: Hepatic PEComa is a rare mesenchymal neoplasm which expresses both melanocytic and myogenic markers. Middle aged females are susceptive to hepatic PEComa, and patients rarely have any specific clinical presentation. It's difficult to make a correct diagnosis before operation. The diagnosis finally depends on the pathological examination. Surgical resection and close follow-up are the principal methods for the management of hepatic PEComa.