近端型上皮样肉瘤4例临床病理分析

目的:探讨近端型上皮样肉瘤的临床病理特征、诊断要点和鉴别诊断.方法:分析4例近端型上皮样肉瘤的临床病理资料,观察其组织病理学形态及免疫表型特征,对其进行随访.结果:女3例,男1例.发病年龄33～58 岁.位于会阴部2例,胸壁1例,背部1例.临床均表现为无痛性生长的坚实性孤立性结节状肿块.瘤细胞呈圆形或卵圆形,含有均质的嗜酸性胞质,胞核位于一侧,核仁明显,常见横纹肌样细胞,少见肉芽肿样改变.瘤细胞波形蛋白(Vim)、细胞角蛋白(CK)、CD34、上皮膜抗原(EMA)阳性例数分别为4/4、4/4、2/4、1/4,均不表达MyoD1、S-100蛋白、SMA、HMB-45、desmin.2例死于发病后8个月以内,1例发病4个月后出现肝转移灶,12个月死亡,另1例随访14个月仍生存.结论:近端型上皮样肉瘤的组织病理学特征是:瘤细胞有弥漫性深染嗜酸性癌样胞质,核大,空泡状,核仁明显,常见横纹肌样细胞;瘤细胞共同表达Vim和CK,半数病例瘤细胞表达CD34,部分病例瘤细胞表达EMA;诊断需与一些具有上皮样细胞形态的良恶性肿瘤相鉴别;其生物学行为高度恶性.

Proximal-type epithelioid sarcoma:A clinicopathological analysis of 4 cases

Objective:To investigate the pathological charcateristics,clinical and differential dignosis of proximal-type epthelioid sarcoma.Methods:The pathologic and immunophenotypic features of 4 cases of proximal-type epithelioid sarcoma were studied and all the cases were followed up.Results:Three of the patients were female and 1 was male.The age of the patients ranged from 33 to 58.The epithelioid sarcoma grew on the perineal region in two,on the chest wall in one and on the back in one.The tumors were painless subcutaneous firm growing solitary nodules.The tumor cells were round or ovoid,containing eosinophilic cytoplasm.The nucleus was located on one-side with obvious nucleoli.Rhabdoid features were frequently observed,seldom resulting in a granuloma-like alteration.The tumors stained positively for vimentin(4/4),CK(4/4),CD34(2/4),EMA(1/4),while negatively for MyoD_1,S-100 Protein,SMA,HMB-45,and desmin.Two patients died after eight months since presentation,one patient was found liver metastasis after four months and died after twelve months,and one patient was still alive after fourteen months.Conclusions:The histopathological characteristics of proximal-type epithelioid sarcoma are as follows:the tumor cells show diffuse and densely eosinophilic cytoplasm,enlarged vesicular nuclei and prominent nucleoli.Rhabdoid features are frequently observed.All the tumor cells express vimentin and CK.The tumor cells express CD34 in half of the cases and express EMA in part of the cases.More attention should be paid to differentiate this tumor from other benign and malignant tumors exhibiting epithelioid features.The biological behaviour of proximal-type epithelioid sarcoma is highly maliganance.