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外周性原始神经外胚层肿瘤(Peripheral primitiveneuroectrodermal tumors,简称PNET)是一种比较少见的来源于周围神经的原始外胚层恶性肿瘤。早在1918年,Stout就对其形态特征作了首次描述,直到近60年后,Nesbitt和Vidone才对该瘤有了较为详细的观察,1993年WHO正式确定为PNET。随着免疫组化、电子显微镜及分子生物学技术的发展,人们对PNET有了更加深入的认识,复习近年来的研究文献,对PNET的临床病理组织学特点作一综述。
Peripheral primitive neuroextrodermal tumors (PNETs) are a relatively rare form of primary ectodermal malignant tumors of the peripheral nerve. As early as 1918, Stout made the first description of its morphological features. Nesbitt and Vidone did not observe the tumor until 60 years later. The WHO formally identified it as PNET in 1993. With the development of immunohistochemistry, electron microscopy and molecular biology techniques, people have more in-depth understanding of PNET, reviewed the research literature in recent years, and reviewed the clinicopathological features of PNET.