De Tommasi A, De Tommasi C, Occhiogrosso G, Cimmino A, Parisi M, Sanguedolce F, Ciappetta P
Department of Neurosurgery, University of Bari, Italy.
Eur J Neurol. 2006 Mar;13(3):240-3. doi: 10.1111/j.1468-1331.2006.01183.x.
Spinal primitive neuroectodermal tumors (PNET) are very rare tumors, and intramedullary localization is even less common. Indeed, amongst the 23 cases of the spinal PNET described in the literature, only eight cases had an intramedullary localization. Following the WHO 2000 classification, PNETs have been considered embryonal tumors composed of undifferentiated neuroepithelial cells with a capacity of differentiation into different cellular lines, such as astrocytic, ependymal, melanotic and muscular. They have been considered to arise from a neoplastic transformation of primitive neuroepithelial cells, thereby making their presence possible in any part of the central nervous system. The optimal treatment for these malignant tumors is not yet clear, although, over the years, radiotherapy has been considered the best treatment for spinal PNETs. The described case is that of a 38-year-old man with a primary intra-extramedullary PNET, treated by laminectomy, open biopsy and chemotherapy. The patient, 18 months after the onset of his symptomatology, died without cerebral tumor involvement.
脊髓原始神经外胚层肿瘤（PNET）非常罕见，髓内定位更为少见。事实上，在文献中描述的23例脊髓PNET病例中，只有8例为髓内定位。按照世界卫生组织2000年的分类，PNET被认为是由未分化神经上皮细胞组成的胚胎性肿瘤，具有分化为不同细胞系的能力，如星形细胞、室管膜细胞、黑色素细胞和肌肉细胞。它们被认为起源于原始神经上皮细胞的肿瘤性转化，因此有可能出现在中枢神经系统的任何部位。这些恶性肿瘤的最佳治疗方法尚不清楚，尽管多年来放疗一直被认为是脊髓PNET的最佳治疗方法。所描述的病例是一名38岁男性，患有原发性髓内外PNET，接受了椎板切除术、开放活检和化疗。该患者在出现症状18个月后死亡，未发生脑肿瘤转移。
