Clinico-pathologic analysis of 12 cases of epithelioid hemangioendothelioma / 临床与实验病理学杂志

ABSTRACT

Purpose To study the clinicopathologic features,immunophenotypes,diagnosis and differential diagnosis of epithelioid hemangioendothelioma (EHE).Methods Histopathology,clinical features and immunophenotypes of 12 cases of EHE which diagnosed by resection and core needle biopsy were analyzed retrospectively.These patients were followed-up.Results There were 9 males and 3 females,aged from 30 to 69 years,with an average age of 43.5 years.Among 12 EHEs,there were 8 cases in liver,3 cases in bone,and 1 case in lung.Histopathologically,the tumor cells were round and oval epithelioid arranged in single,nests,and cords.Tumor cells had abundant eosinophilic cytoplasm that contained vacuoles and erythrocyte in the tumor cytoplasm.The stroma showed myxoid change.Sparse calcification could be seen in some EHEs.Immunophenotype,tumor cells showed strong positive for CD34,CD31 and factor Ⅷ related antigen (FⅧRAg).Most EHEs are alive with tumor.1 case recurred in 6 months after surgery.1 patient died at 100 months postoperation.3 cases were lost follow-up.Conclusion EHE is a very rare low-grade malignant vascular neoplasm especially for arising from solid organ.Precise diagnosis must depend on surgical histopathology and molecular biology test of WWTR1-CAMTA1 fusion gene.