腹膜后原始神经外胚层肿瘤病例报道并文献复习

Case Report and Literature Review:Retroperitoneal Primitive Neuroectodermal Tumor

目的提高的腹膜后原始神经外胚层肿瘤(retroperitoneal primitive neuroectodermal tumorrPNET)认识和诊断水平。方法报告1例腹膜后腹膜后原始神经外胚层肿瘤患者的临床资料,CT及病理表现,并复习相关文献。结果该病临床无特异性,CT表现为恶性肿瘤征象,但无明显特异性,病理具备恶性小圆细胞肿瘤的特征,免疫组化弥漫表达CD99。结论腹膜后PNET属于恶性小圆细胞肿瘤,预后非常差,临床及CT表现无明显特征,定性诊断仍依赖于病理检查,但CT能较准确描述肿瘤的大小、形态及毗邻关系,对手术有指导意义。

Objective To enhance the understanding and the correct diagnosis of retroperitoneal primitive neuroectodermal tumorr（rPNET）. Methods To report a case of rPNET with the clinic data, CT and pathological findings, and review the related literatures. Results The disease has not specific clinical feature, CT manifestations shows mailignant signs, pathology has the characteristics of malignant small round cell tumor, diffuse expression of CD99, and varying degrees of expression of NSE, Syn, Vim, S2100. Conclusion The rPNET are malignant small round celt tumor, the prognosis is very bad, The clinic and imaging of rPNET lack of specialties,its final diagnosis was still relies on pathological examination,but CT can better displayed the size ,range and adjacent tissue relationship of rPNET,it could be confer a guiding signifcance for the surgery.