1. Hepatic perivascular epithelioid cell tumor in three patients
- Author
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Chuang-Yong Zhang, Xiao Li, Bao-Bin Hao, Feng Zhang, Ye Fan, Jianhua Rao, Xinzheng Dai, and Yan Leng
- Subjects
Adult ,Male ,Pathology ,medicine.medical_specialty ,Time Factors ,Perivascular Epithelioid Cell Neoplasms ,medicine.medical_treatment ,Biopsy ,CD34 ,Adipose tissue ,Perivascular Epithelioid Cell ,03 medical and health sciences ,0302 clinical medicine ,Predictive Value of Tests ,Biomarkers, Tumor ,Medicine ,Hepatectomy ,Humans ,Ultrasonography ,Hepatology ,medicine.diagnostic_test ,business.industry ,Liver Neoplasms ,Gastroenterology ,Soft tissue ,Middle Aged ,Immunohistochemistry ,Treatment Outcome ,030220 oncology & carcinogenesis ,Predictive value of tests ,030211 gastroenterology & hepatology ,Female ,Neoplasm Recurrence, Local ,business ,Tomography, X-Ray Computed - Abstract
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 PEComas 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.
- Published
- 2016