Kumar Raj, Reddy S J, Wani A A, Pal L
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Pediatr Neurosurg. 2007;43(1):1-6. doi: 10.1159/000097517.
Primary spinal primitive neuroectodermal tumor (PSPNET) is extremely rare and only 25 cases have been reported in the world literature so far. Three patients of 8, 9 and 18 years of age, who presented with variable grades of neurological deficit were diagnosed as having a dorsal intramedullary lesion, a holocord lesion and cervical extradural tumor with extraspinal extension, respectively, and were operated at our institute. The histopathology of all 3 children revealed PNET. The clinical course, image characteristics and outcome of the 3 children are described, and the relevant literature is reviewed. The following conclusions were drawn from the present study and review of the literature. PNET may manifest itself as a primary lesion of the spine unlike the more common drop metastases from an intracranial lesion. PSPNET may be intramedullary, intradural and extradural with variable extraspinal extension. PSPNET may present as holocord intramedullary lesion, an entity which has not been described earlier. These lesions have a short history, significant neurological deficits and rapid course of illness. PSPNET, though an established entity, did not find a place in the WHO 2000 classification of CNS tumors. Hence its status has to be defined.
原发性脊髓原始神经外胚层肿瘤（PSPNET）极为罕见，迄今为止世界文献中仅报道了25例。我院收治了3例分别为8岁、9岁和18岁的患者，他们均表现出不同程度的神经功能缺损，分别被诊断为髓内背侧病变、全脊髓病变以及伴有椎体外延伸的颈段硬膜外肿瘤，并接受了手术治疗。所有3例患儿的组织病理学检查均显示为原始神经外胚层肿瘤（PNET）。本文描述了这3例患儿的临床病程、影像特征及预后，并对相关文献进行了综述。通过本研究及文献回顾得出以下结论。与更常见的颅内病变的播散性转移不同，PNET可能表现为脊柱的原发性病变。PSPNET可位于髓内、硬膜内及硬膜外，并伴有不同程度的椎体外延伸。PSPNET可能表现为全脊髓髓内病变，这一实体此前尚未见描述。这些病变病程短，神经功能缺损严重，病情进展迅速。尽管PSPNET已是一个明确的实体，但在世界卫生组织2000年的中枢神经系统肿瘤分类中并未提及。因此，其地位有待明确。
