口腔颌面部Ewing肉瘤/原始神经外胚层瘤的临床病理学研究

Clinicopathological study of Ewing′s sarcoma/primitive neuroectodermal tumor in oral and maxillofacial region

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.

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 ①The 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.