Hepatic perivascular epithelioid cell tumor in three patients

• Published in: Hepatobiliary & pancreatic diseases international 2016-12, Vol.15 (6), p.660-664
• Main Authors: Hao, Bao-Bin, Rao, Jian-Hua, Fan, Ye, Zhang, Chuang-Yong, Dai, Xin-Zheng, Li, Xiao, Leng, Yan, Zhang, Feng
• Format: Article
• Language: English
• Subjects: Adult; Biomarkers, Tumor - analysis; Biopsy; diagnosis; Endocrinology & Metabolism; Female; Gastroenterology and Hepatology; Hepatectomy; Humans; Immunohistochemistry; Liver Neoplasms - chemistry; Liver Neoplasms - pathology; Liver Neoplasms - surgery; Male; Middle Aged; Neoplasm Recurrence, Local; Perivascular Epithelioid Cell Neoplasms - chemistry; Perivascular Epithelioid Cell Neoplasms - pathology; Perivascular Epithelioid Cell Neoplasms - surgery; perivascular epithelioid cell tumor, hepatic; Predictive Value of Tests; recurrence; Time Factors; Tomography, X-Ray Computed; Treatment Outcome; Ultrasonography
• ISSN: 1499-3872
• DOI: 10.1016/S1499-3872(16)60077-2

Perivascular epithelioid cell tumor （PEComa） is a rare, soft tissue tumor that can occur in various locations. The present report included three patients （one male and two females; age range, 25-51 years） with hepatic PEComas. The collected data included the clinical manifestations, diagnosis, management, treatment, and prognosis. Since it is difficult to diagnose hepatic PEComas by imaging, the patients were diagnosed by tumor tissue examination such as immunohistochemistry, which was positive for HMB-45, Melan-A, and SMA on all slides. The tumor was composed of diverse tissues including smooth muscle, adipose tissue, and thick-walled blood vessels. During the follow-up period, one of the tumors was malignant （double-positive for CD34 and Ki-67） and recurred 3 months after surgery. In addition, malignant hepatic PECo- mas were reviewed in the literature, indicating that the majority of hepatic PEComas are benign, but few hepatic PEComas exhibit malignant behaviors in older female patients （〉50 years of age） with abdominal discomfort and pain, larger tumor size （〉10 cm）, or positive staining for CD34 and Ki-67. In conclusion, there is no effective method to diagnose PEComas. Currently, the diagnosis of PEComas depends on immunohistochemical staining. Tumor resection and close follow-up are the principal methods for the management of PEComas.