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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 improve the understanding of clinicopathological features of Ewing’s sarcoma / primitive neuroectodermal tumor (Ewing’s sarcoma / PNET) in oral and maxillofacial region and to reduce the misdiagnosis. Methods A total of 15 cases with Ewing’s sarcoma / PNET diagnosed from January 1970 to December 2004 were collected from the Department of Pathology, West China School of Stomatology, Sichuan University from January 1970 to December 2004. Their ages ranged from 1 to 49 years (average 14.5 years). The main manifestations were swelling, Radiographically visible bone destruction. The histological features were analyzed and immunohistochemical staining was performed by LSAB. The antibodies were CD99 (12E7), vimentin, neuron specific enolase (NSE), S-100, Syn, CD45 (LCA) and desmin. Results ① Histological features: Tumors were mainly composed of dense small cells, diffusely distributed, fibrous cords were separated into irregular patches, visible daisy-like structure, some cells containing glycogen. ② Immunohistochemical staining: Seven cases of CD99 and vimentin were positive, CD45 and desmin were all negative; four cases of S-100 positive, three NSE positive, one Syn positive, and two cases of NSE, S-100, Syn are negative. Conclusion Ewing’s sarcoma / PNET is extremely rare in oral and maxillofacial region, mostly adolescents. Immunohistochemistry can be used to diagnose Ewing’s sarcoma. The high expression of p30 / 32MIC2 is valuable for the diagnosis.