摘要
目的探讨上皮样肉瘤(ES)的临床病理学特征、诊断和鉴别诊断。方法收集49例上皮样肉瘤的临床病理学资料,分析其临床和病理学特征、免疫表型及鉴别诊断。结果 49例患者年龄10～83岁,平均年龄38.8岁,男女之比为1.9∶1。"经典型"是上皮样肉瘤的常见类型,多发生于四肢尤其是上肢的软组织;"近心型"上皮样肉瘤罕见,多发生于中线软组织。上皮样肉瘤具有多结节状生长,细胞呈上皮样分化,中心性坏死和肉芽肿样结构的组织学特征,需与多个肿瘤及非肿瘤性病变鉴别。免疫组化vimentin、CK、EMA和CD34(+)。染色体22q基因表达缺失具有较高的遗传学特异性。结论上皮样肉瘤罕见,恶性程度相对较低,但复发率高,诊断应结合临床、组织学、免疫表型及遗传学特征综合分析。
Objective To investigate the clinicopathologic features,immunohistochemistry and differential diagnosis of epithelioid sarcoma（ES）.Methods The clinical and pathological features,diagnosis and differential diagnosis were analyzed in 49 cases of epithelioid sarcoma.Results The age of patients ranged from 10 to 83 years,and the mean age was 38.8 years.The male-to-female ratio was 1.9∶1.Classic-type was the most common subtype of ES,conventionally involving the soft tissues in the distal extremities.A rare subtype of ES involving axial soft tissues named proximal type.A multinodular mass,epithelioid tumor cells,central necrosis and granulomatous lesions were the histological features of ES.Many tumors and nontumorous lesions should be differentiated.Immunohistochemically,vimentin,CK,EMA and CD34 staining were positive.There was a high genetic specificity in chromosomal abnormalities in 22q region.Conclusions ES is a rare,relatively low grade tumor with a higher recurernce rate.A correct diagnosis relies on clinical,histological,immunohistochemical features and genetic conditions.
出处
《诊断病理学杂志》
CSCD
2011年第6期407-410,共4页
Chinese Journal of Diagnostic Pathology
关键词
上皮样肉瘤
临床病理
诊断
鉴别诊断
Epithelioid sarcoma
Pathology
Diagnosis
Differential diagnosis
