Isotalo P A, Agbi C, Davidson B, Girard A, Verma S, Robertson S J
Department of Laboratory Medicine, Ottawa Hospital, and University of Ottawa, Ontario, Canada.
Hum Pathol. 2000 Aug;31(8):999-1001. doi: 10.1053/hupa.2000.16532.
Primitive neuroectodermal tumors (PNETs) are aggressive neoplasms composed predominantly of undifferentiated cells that show evidence of neural differentiation. Although their classification has been controversial, PNETs are well recognized primary tumors of both central and peripheral nervous systems. PNETs must be distinguished from other round-cell tumors, including Ewing's sarcoma, lymphoma, rhabdomyosarcoma, and small cell carcinoma. Intraspinal PNETs are rare neoplasms that are usually metastatic in origin. We describe the eighth reported primary PNET of the cauda equina that developed in a 52-year-old man with no significant medical history. The tumor was characterized by Homer-Wright rosettes and immunoreactivity for CD99, glial fibrillary acidic protein, neuron-specific enolase S100, and synaptophysin. The anatomic location of primary intrathecal PNETs is important as those arising in the spinal cord develop in the central nervous system, whereas those arising in the cauda equina develop in the peripheral nervous system. The histogenesis of intrathecal PNETs may be multifactorial.
原始神经外胚层肿瘤（PNETs）是侵袭性肿瘤，主要由未分化细胞组成，这些细胞显示出神经分化的证据。尽管其分类一直存在争议，但PNETs是中枢神经系统和周围神经系统公认的原发性肿瘤。PNETs必须与其他圆形细胞肿瘤相鉴别，包括尤因肉瘤、淋巴瘤、横纹肌肉瘤和小细胞癌。脊髓内PNETs是罕见肿瘤，通常起源于转移性肿瘤。我们描述了第八例报道的马尾原发性PNET，该肿瘤发生在一名无重大病史的52岁男性身上。肿瘤的特征是霍纳-赖特玫瑰花结以及对CD99、胶质纤维酸性蛋白、神经元特异性烯醇化酶S100和突触素的免疫反应性。原发性鞘内PNETs的解剖位置很重要，因为发生在脊髓的肿瘤在中枢神经系统中发展，而发生在马尾的肿瘤在周围神经系统中发展。鞘内PNETs的组织发生可能是多因素的。
