Abstract:

Hu Bin*, Wu Kan, Yao Xu，Song Hao, Wen Sijian, Chen Hao, Sun Jianfang. *Department of Dermatology, Wuhan No.1 Hospital, Wuhan 430022, China Corresponding authors: Chen Hao, Email: ch76ch@163.com; Sun Jianfang, Email: sunjf57@163.com 【Abstract】 Objective To better characterize the clinical and histopathological manifestations, diagnosis and differential diagnosis of epithelioid sarcoma （ES）. Methods Clinical data were collected from nine patients with ES diagnosed and treated at the Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College from 2000 to 2014. The clinical and histopathological manifestations, immunophenotype，treatment and prognosis of ES were reviewed retrospectively. Results The median age at onset of ES was 34.5 years in these patients, and the average disease duration was 8.3 years. Lesions began on the extremities in 8 patients, and manifested as nodules, ulceration and lymphedema. Histopathological examination showed that tumor cells mainly consisted of spindle cells and epithelioid cells, some of which were in a palisade arrangement with central necrosis. Cytokeratin, epithelial membrane antigen （EMA） and vimentin were coexpressed by tumor cells. Recurrence was observed at the original site in 6 patients after lesion resection, and pulmonary metastasis occurred in 4 patients. Five patients were followed up and two of them died of pulmonary metastasis. Conclusions Local recurrence is frequent in patients with ES after lesion removal. Lymph node and pulmonary metastases of ES are common, and pulmonary metastasis is usually associated with a poor prognosis.