【摘要】 目的提高对口腔颌面部Ewing肉瘤/原始神经外胚层瘤(Ewing肉瘤/PNET)的临床病理特征的认识,减少误诊。方法收集四川大学华西口腔医学院病理科1970年1月至2004年12月临床病理诊断符合Ewing肉瘤/PNET的病例共15例,年龄1~49岁,平均14·5岁,主要表现为肿胀,影像学可见骨质破坏。结合文献对其组织学特征进行分析,并行免疫组化LSAB法染色,抗体为CD99(12E7)、波形蛋白、神经元特异性烯醇化酶(NSE)、S-100、突触素(Syn)、CD45(LCA)及结蛋白。结果①组织学特征:肿瘤主要由密集的小细胞组成,弥漫分布,纤维性条索将其分隔成不规则片块状,可见菊形团样结构,部分细胞含糖原。②免疫组化标记:所选7例CD99及波形蛋白均阳性,CD45、结蛋白均阴性;S-100阳性4例,NSE阳性3例,Syn阳性1例,2例对NSE、S-100、Syn均为阴性。结论口腔颌面部Ewing肉瘤/PNET极为少见,多为青少年,免疫组化可辅助诊断,p30/32MIC2高水平表达对确诊有价值。更多还原

【Abstract】 Objective To study the clinicopathological features and diagnostic criteria of Ewing′s sarcoma/primitive neuroectodermal tumor(Ewing′s sarcoma /PNET) in oral and maxillofacial region. Methods There were 15 patients with Ewing′s sarcoma /PNET in the last 35 years at our hospital, aged 1-49 years and mean 14.5 years. The most common manifestation was swelling of the affected region. The cases were analyzed and histological and immunohistochemical studies were also conducted to examine CD99(12E7),Vimentin,NSE,S-100,Syn,CD45(LCA),desmin. Results ① The most common histological pattern of Ewing′s sarcoma / PNET was a lobular arrangement of uniform, small, hyperchromatic cells in a fibrous background. Some of these tumors were rich in cytoplasmic glycogen. ② Of the 7 cases,IHC was positive for CD99(12E7) and Vimentin and negative for lymphoid (CD45), muscle (desmin) markers. S-100 was positive in 4 cases and NSE negative in 3,Syn positive in 1. NSE,S-100,Syn were all negative in 2 cases. Conclusions Ewing′s sarcoma /PNET is more common in teenagers and young people. Immunohistochemistry is essential to distinguish Ewing′s sarcoma /PNET from other small round cell tumors. Immunohistochemistry is useful in the diagnosis.更多还原