上皮样肉瘤九例分析

Clinicopathological analysis of nine cases of epithelioid sarcoma

目的探讨上皮样肉瘤临床及组织病理诊断及鉴别诊断的要点。方法回顾性分析中国医学科学院皮肤病医院2000-2014年诊治的9例上皮样肉瘤患者的临床表现、组织病理学、免疫组化、治疗方法及预后。结果9例患者中位发病年龄34．5岁，就诊时平均病程8-3年。8例始发于肢端，表现为结节、溃疡及淋巴水肿。组织病理示肿瘤细胞主要由上皮样细胞和梭形细胞构成，可呈栅栏状排列，中央有坏死，肿瘤细胞共表达细胞角蛋白、上皮膜抗原、波形蛋白。6例患者皮损切除后原位复发，4例伴肺部转移。随访5例，其中2例死于肺转移。结论上皮样肉瘤切除后局部复发率高，容易发生淋巴结和肺部转移，发生肺部转移后预后不佳。

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 ceils mainly consisted of spindle ceils 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.