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目的:提高对鼻咽部原始神经外胚层肿瘤(PNET)病理诊断的认识,减少误诊。方法:报告1例鼻咽部原始神经外胚层肿瘤患者的临床资料、病理特征,并复习相关文献。结果:该病临床无特异性,病理特征有肿瘤细胞体积较小,呈圆形或卵圆形,间质较少,有聚集成团倾向,肿瘤弥漫表达CD99,并不同程度地表达NSE、Syn、Vim、S-100等。结论:PNET是一种预后非常差的恶性小圆细胞肿瘤,认识其临床病理特点及免疫组化表型对于该恶性肿瘤的诊断及临床治疗意义重大。
Objective: To improve the understanding of pathological diagnosis of nasopharyngeal primitive neuroectodermal tumor (PNET) and reduce the misdiagnosis. Methods: One case of nasopharyngeal primitive neuroectodermal tumor patients reported clinical data, pathological features, and review the relevant literature. Results: The clinical features of this disease were nonspecific. The pathological features of the tumor were small, round or oval in shape, less interstitial mass, tendency to agglomerate into clusters, diffusely express CD99 in tumor, and express NSE, Syn , Vim, S-100 and so on. Conclusion: PNET is a malignant small cell tumor with very poor prognosis. To understand its clinicopathological features and immunohistochemical phenotype is of great significance for the diagnosis and clinical treatment of this malignant tumor.