摘要
目的探讨近端型上皮样肉瘤的临床病理特点、免疫表型及诊断和鉴别诊断。方法分析了6例近端型上皮样肉瘤的临床表现、影像学、组织学及免疫组化表达特点。免疫组化染色采用SP法。所用一抗包括CK(AE1/AE3)、EMA、vimentin、desmin、S-100、CA125、β-catenin、HMB45、CD117、CD99、CD34、MSA等。结果男性5例女性1例,年龄范围32～74岁(平均56岁)。临床上表现为近期迅速增大的软组织肿块并伴有疼痛及相应的功能障碍,肿块位于深筋膜或肌肉内。CT显示为软组织肿块影,密度与肌肉相近并有明显的不均匀强化;超声检查为实性低回声肿物,肿块内可见血流信号;MRI-T1加权显示等信号,MRI-T2加权显示高信号。肿块呈多结节状,缺乏包膜,直径3～25cm(平均7cm);切面灰白色及灰红色,质地偏软呈鱼肉样,灰白色区域质地偏韧。在组织学上,肿瘤主要由明显异型的上皮样细胞组成,上皮样细胞呈中等大小,或卵圆形,核仁明显,核分裂象易见,胞质嗜酸性,丰富,细胞界限不清,少部分细胞呈梭形并与上皮样细胞间有移行,此外还有部分瘤细胞为横纹肌样细胞,表现为核偏位,核形不规则,核仁不明显,胞质丰富,嗜酸性,并有核内包涵体;肿瘤坏死明显并侵及横纹肌、骨等周围组织。随访结果:3例病人术后复发,2例患者死于广泛转移。免疫组化染色显示主要表达EMA(6/6)、vimentin(6/6)、AE1/AE3(5/6)、CD99(5/6)、β-catenin(6/6)、CD34(4/6),而HMB45、S-100、CD117、CD31等均为阴性。结论近端型上皮样肉瘤是一中度恶性肿瘤,其组织学形态主要表现为上皮样细胞和横纹肌样细胞,需与具有类似细胞形态的多种肿瘤鉴别;结合免疫表型,对诊断很有帮助。
Purpose To study the clinicopathological features,immunophenotype and differential diagnosis of proximal epithelioid sarcoma （PES）.Methods The clinical,radiological and pathological features were reviewed in 6 cases of PES.Results The patients consisted of five males and one female with ages ranging from 32 to 74 years （mean 56 years）.Clinically,the tumor mass were progressively-growing solitary nodules with the fuctional disorder and pain.The sites of the tumor involvement were buttock （1 case）,thigh （2 cases）,iliac spine （1 case）,lumbar spine （1 case） and anterior sacrum （1 case）.Histologically,the tumors were mainly consisted of epithelioid cells with round or oval nuclei,prominent nucleoli and eosinophilic cytoplasm,some tumor cells appeared rhabdoid features.Immunohistochemically,the tumor cells were positive for vimentin （6/6）,CK （5/6）,EMA （6/6）,β-catenin （6/6）,CD99 （5/6）,and CD34 （4/6）,but negative for S-100 protein,HMB 45,CD 117 and CD 31.Conclusion PES is a distinctive moderate grade malignant tumor with prominent epithelioid and rhabdoid cells appearance.The differential diagnoses include the tumors that have similar cellular appearance with PES,and immunohistochemical staining is useful for diagnosis and differential diagnosis.
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2010年第1期73-76,共4页
Chinese Journal of Clinical and Experimental Pathology
