外周型原始神经外胚层瘤临床病理分析

Clinicopathologic analysis of peripheral primitive neuroectodermal tumors

目的探讨外周型原始神经外胚层瘤(p PNET)的临床病理及免疫组织化学特点和诊断标准。方法采用常规苏木精-伊红染色,免疫组织化学法,结合影像学表现,对12例p PNET进行观察分析。结果 12例患者临床表现为增长迅速的局部肿块,伴有疼痛,以及肿块压迫所引起的症状。影像学表现缺乏特异性。镜下肿瘤主要由小圆细胞构成,3例可见Homer-Wright菊形团形成;免疫组织化学结果显示CD99阳性率为100%(12/12),NSE和Syn阳性率较高,分别为92%(11/12)和75%(9/12),Vim的阳性表达率为50%(6/12)。结论 p PNET是一种少见的高度恶性小圆细胞肿瘤,病理形态学特征及免疫组织化学标记有助于p PNET的诊断和鉴别诊断。

Objective To investigate the clinicopathologic and immunohistochemical characteristics of peripheral primitive neuroectodermal tumors （pPNET） and its diagnostic criteria. Methods 12 cases of pPNET were observed and analyzed by the HE staining, immmunohistochemistry, and imaging examination. Results 12 patients with pPNET presented progressively growing masses, local pain, and symptoms caused by mass compression. Imaging findings lacked specificity. Under the microscope, tumors consisted of small round cells and Homer-Wright rosettes were observed in 3 cases. Results of immunohistochemistry showed that the positive rate of CD99 was 100% （12/12）. Positive rates of NSE and Syn were high, i. e. 92% （11/12） and 75% （9/12）, respectively. The positive rate of Vim was 50% （6/12）. Conclusion pPNET is a rare, highly malignant sarcoma. Pathological and morphological characteristics and immunohistochemistry labels are helpful for the diagnosis and differential diagnosis of pPNET.