近端型上皮样肉瘤5例临床病理分析

目的 探讨近端型上皮样肉瘤(PES)的临床病理特点、免疫表型、诊断及鉴别诊断.方法 收集5例PES患者的临床资料,观察其组织病理学形态、免疫组化特征并进行随访.结果 5例PES中男性3例,女性2例,发病年龄28～54岁,分别位于头部、臀部、腹股沟、胸壁、胸椎.临床均表现为无痛性生长的结节状肿块.镜下肿瘤由上皮样细胞、梭形细胞混合并相互过渡组成,呈结节状或结节融弥漫合成片状,中央常见坏死;其中2例周边可见肉芽肿性改变.细胞间见明显的胶原纤维沉积.细胞呈上皮样或梭形,胞质丰富,嗜酸性;细胞核卵圆形、空泡状,核仁明显,一个或多个,核分裂象多见,常见横纹肌样细胞.免疫表型:5例瘤细胞vimentin、CKpan、EMA和β-catenin(+),3例CD34(+),1例desmin(+),2例CD99(+),而S-100、HMB45、melanA、SMA、myogenin、CD31、FⅧ、HHF35和bcl-2均(-).5例PES均行肿物局部切除或扩大切除,其中3例辅以放疗或化疗,5年的随访中均有复发和转移.结论 PES因肿瘤组织主要由上皮样细胞、横纹肌样细胞及梭形细胞构成,形态复杂多样,极易与具有类似形态的多种肿瘤混淆,故其鉴别诊断显得尤为重要.

Diagnosis and differential diagnosis of proximal-type epithelioid sarcoma

Objective To investigate the clinicopathological features,immunophenotype and differential diagnosis of proximal-type epithelioid sarcoma(PES).Methods The pathologic and immunophenotypic features were collected and analyzed with follow-up in 5 cases of PES.Results Three of the patients were male and another two were female.The age of patients ranged from 28 to 54 years.The 5 tumors located at head,buttock,groin,chest wall and dorsal vertebrae,respectively.Clinically,the tumors were painless firm and subcuta...