【摘要】 目的探讨骨和软组织的尤文氏瘤（ＥＷ）与外周性原始神经外胚层肿瘤（ＰＮＥＴ）的诊断及预后。方法对１４例该类肿瘤的病理形态和免疫组化、８例超微结构以及１２例随访情况进行了研究，采用Ｓｃｈｍｉｄｔ的诊断标准划分ＥＷ与ＰＮＥＴ。结果ＥＷ６例，ＰＮＥＴ８例。１４例均呈小细胞形态，６／８例ＰＮＥＴ有Ｈ－Ｗ菊形团，而６例ＥＷ均未见该结构。１２／１４例的Ｏ１３染色阳性，３／６例ＥＷ仅ＮＳＥ一项阳性，而８例ＰＮＥＴ均有神经标记的表达，且５例伴有两项以上的此标记。４例ＰＮＥＴ均有神经内分泌颗粒。１２例ＰＡＳ中３／６例ＥＷ阳性，１／６例ＰＮＥＴ阳性。随访２个月至５年，除３例骨内ＥＷ无瘤生存外，其余均已死亡或转移。结论ＥＷ分化较原始；而ＰＮＥＴ的神经分化较明显，且Ｈ－Ｗ菊形团是ＰＮＥＴ的重要形态特点。本研究提示区分ＥＷ与ＰＮＥＴ具有一定的临床意义。Ｏ１３是诊断ＥＷ与ＰＮＥＴ的有用标记。更多还原

【Abstract】 Objective To study the diagnosis and differentiation of EW and PNET. Methods Fourteen cases of Ewing’s sarcomas (EW) and peripheral primitive neuroectodermal tumors (PNET) were studied by light microscopy, immunohistochemistry and electron microscopy (EM). Twelve cases were followed up. Schmidt criterion was used for the differential diagnosis of EW and PNET. Results There were 6 cases of EW and 8 cases of PNET. six of 8 PNET cases had Homer Wright (H W) rosettes. In this series, 12/14 cases were positive for O 13 (HBA71) staining. NSE was positive in 3 cases of EW. All PNET cases were positive for neural markers, and 5 of them were positive for more than two of these markers. Electronmicroscopically, there were neurosecretory granules (4/4 cases), nerve like protrusions and microfilaments (1/4 case) in PNET. In 3 of 6 EW and 1 of 6 PNET, PAS staining was positive. During the follow up period from 2 months to 5 years, 3 cases with intraosseous EW remain alive and free of tumor. The remaining patients are dead or having their tumors metastasized. Conclusion EW is more primitive in cell differentiation, while PNET has more neural differentiation. The presence of H W rosettes is an important morphologic feature of PNET. To differentiate EW from PNET is of clinical significance O 13 is a useful marker for the diagnosis of EW/PNET.更多还原