Diagnosis and surgical treatment of hepatic perivascular epithelioid cell carcinoma / 中华消化外科杂志

ABSTRACT

Objective To investigate the clinical featurcs of hepatic perivascular epithelioid cell carcinoma (PEComa) and the experiments in the surgical treatment of PEComa.Methods The clinical data of 16 patients with hepatic PEComa who received surgical treatment at the First Affiliated Hospital of Nanjing Medical University from January 2008 to January 2012 were retrospectively analyzed.The incidence,clinical manifestations,imagiong characteristics,surgical outcomes and pathological manifestations of this disease were analyzed.B sonography,hepatic function test,tumor markers test and epigastric computed tomography (CT) were applied to detect tumor recurrence and metastasis.The follow-up was ended in December 2012.Result Middle aged and female patients took large part of the patients.Of the 16 patients,8 had no subjective symptoms,and other patients had discomfort in the right upper quadrant,pain or tenderness of the liver.All the tumors were solitary,and most of them located at the right liver (11 tumors were in the right liver,4 in the left liver,1 in the caudate lobe).No specific features were detected by preoperative B sonography,while inhomogeneous low density in the tumor region was detected on by CT.All the patients received partial liver resection without morbidity and morality.The results of immunohistochemistry showed that thc expressions of HMB-45,Melan-A and vascular smooth muscle actin were positive.The mean time of follow-up was 27.9 months (range,9.0-46.0 months),no tumor recurrence and death was observed during the follow-up.Conclusions Middle aged females are susceptive to hepatic PEComa,and patients have no specific clinical presentations.Preoperative CT examination is helpful for differential diagnosis of PEComa,and partical hepatectomy can achieve satisfactory short-term clinical outcomes.