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目的探讨上皮样肉瘤(ES)的临床、病理学特征及鉴别诊断要点。方法收集17例上皮样肉瘤患者资料,观察和分析其临床和组织病理学特征,通过免疫组化方法分析其细胞角蛋白(CK)、上皮膜抗原(EMA)、波形蛋白(Vimentin)、S-100蛋白、CD34及平滑肌肌动蛋白(SMA)的表达。结果 17例患者中,男性13例,女性4例,男性多见。平均年龄35.6岁。远端型6例,近心型11例。两型病理形态不同,但免疫组化表达一致,均表达CK、EMA、Vimentin和CD34。初次手术后1年、3年复发及转移率分别为50.0%(8/16)和87.5%(14/16)。结论 ES是一种极易局部复发和远处转移的恶性肿瘤,免疫组化有助于诊断及鉴别诊断。根治性手术是首选治疗方案,ES易发生淋巴结转移,区域淋巴结清扫是必要的。
Objective To investigate the clinical and pathological features and differential diagnosis of epithelioid sarcoma (ES). Methods The data of 17 patients with epithelioid sarcoma were collected. The clinical and histopathological features were observed and analyzed. The expressions of cytokeratin (CK), epithelial membrane antigen (EMA), Vimentin, S- 100 protein, CD34 and smooth muscle actin (SMA) expression. Results Of the 17 patients, 13 were males and 4 were females, more common in males. The average age of 35.6 years old. 6 cases of distal type, 11 cases of proximal type. Two different pathological forms, but immunohistochemical expression consistent, all expressed CK, EMA, Vimentin and CD34. One year after initial surgery, the 3-year recurrence and metastasis rates were 50.0% (8/16) and 87.5% (14/16), respectively. Conclusion ES is a malignant tumor that can easily local recurrence and distant metastasis. Immunohistochemistry is helpful for the diagnosis and differential diagnosis. Radical surgery is the treatment of choice, ES prone to lymph node metastasis, regional lymph node dissection is necessary.