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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。
Objective To investigate the clinicopathological features, immunophenotype, diagnosis and differential diagnosis of proximal type epithelioid sarcoma (PES). Methods The clinical data of 5 patients with PES were collected and their histopathological features and immunohistochemical characteristics were observed and followed up. Results There were 3 males and 2 females in 5 cases of PES. The age of onset was 28-54 years. They were located in the head, buttocks, groin, chest wall and thoracic spine respectively. Clinical manifestations of painless growth of nodular mass. Microscopic tumor by the epithelioid cells, spindle cells mixed with each other and transitional composition of nodular or nodular fusion diffuse sheet, the central common necrosis; of which 2 cases of granulomatous changes seen around. Interstitial cell see obvious collagen deposition. Cells were epithelioid or spindle shape, rich in cytoplasm, eosinophilic; nucleus oval, vacuolar, prominent nucleoli, one or more, mitotic common more common rhabdomyosarcoma cells. Immunophenotype: Five cases of vimentin, CKpan, EMA and β-catenin (+), three cases of CD34 (+), one case of desmin (+), two cases of CD99 (+), and S-100, HMB45, melanA , SMA, myogenin, CD31, FⅧ, HHF35 and bcl-2 (-). Five patients underwent partial resection or extended resection of the tumor. Three of them were treated with radiotherapy or chemotherapy and had recurrence and metastasis at 5-year follow-up. Conclusion Because of the tumor tissue mainly composed of epithelial cells, striated muscle cells and spindle cells, the morphology is complex and diverse, and easily confused with a variety of tumors with similar morphology, so the differential diagnosis is particularly important.