Deme S, Ang L C, Skaf G, Rowed D W
Division of Neurosurgery, Sunnybrook Health Science Center, Toronto, Ontario, Canada.
Neurosurgery. 1997 Dec;41(6):1417-20. doi: 10.1097/00006123-199712000-00040.
Primary intraspinal primitive neuroectodermal tumors (PNETs) are rare. We report a case and review the literature.
A 22-year-old woman presented with rapidly progressive paraparesis and neurogenic bladder.
Preoperative computed tomography myelograms revealed a complete block at T12-L1, consistent with an intramedullary lesion. An urgent operation was performed with gross total tumor removal. The pathological findings were consistent with a PNET. Recurrence was noted within 10 weeks of surgery and has been somewhat responsive to chemotherapy and radiotherapy thus far. A review of the English literature shows that only 13 cases of primary intraspinal PNETs have been reported to date, and the present case is the second one in which the tumor was purely intramedullary. Most of the reported patients survived less than 2 years.
Primary intraspinal PNETs are rare tumors and carry a poor prognosis.
原发性脊髓内原始神经外胚层肿瘤（PNETs）较为罕见。我们报告一例并回顾相关文献。
一名22岁女性出现快速进展的双下肢轻瘫和神经源性膀胱。
术前计算机断层扫描脊髓造影显示T12 - L1水平完全梗阻，与髓内病变相符。紧急进行了手术，肿瘤全切。病理结果符合PNET。术后10周内出现复发，目前对化疗和放疗有一定反应。对英文文献的回顾表明，迄今为止仅报道了13例原发性脊髓内PNETs，本病例是第二例肿瘤为纯髓内的病例。大多数报道的患者存活时间不足2年。
原发性脊髓内PNETs是罕见肿瘤，预后较差。
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