Venkataraman Sundar, Pandian Chelladurai, Kumar Suresh A
Department of Neurosurgery, Madras Institute of Neurology Madras Medical College, Chennai - 600003, INDIA.
Ann Neurosci. 2013 Apr;20(2):80-2. doi: 10.5214/ans.0972.7531.200211.
Primitive neuroectodermal tumours (PNET) are aggressive childhood malignancies and offer a significant challenge to treatment. A two years old female child presented with weakness both lower limbs. Preoperative MRI of the spine and paravertebral regionIso - hyper intense posterior placed extradural lesion, non contrast enhancing from D11-L2 levels with cord compression D9 to L3 laminectomy done. Granulation tissue found from D11 to L2. with cord compression. The granulation tissue removed in toto. The pathological findings were consistent with PNET. Post operative neurological improvement was minimal. Cranial screening ruled out any intracranialtumour. Hence a diagnosis of primary spinal PNET was made. A review of the literature shows that only 19 cases of primary intraspinal PNETs have been reported to date and the present case extradural in location. Primary intraspinal PNETs are rare tumors and carry a poor prognosis.
原始神经外胚层肿瘤（PNET）是侵袭性儿童恶性肿瘤，对治疗构成重大挑战。一名两岁女童出现双下肢无力。术前脊柱和椎旁区域的MRI显示，硬膜外病变位于后方，等T1加权像和T2加权像呈高信号，从胸11至腰2水平无强化，脊髓受压，行胸9至腰3椎板切除术。在胸11至腰2发现肉芽组织，伴有脊髓受压。将肉芽组织全部切除。病理结果与PNET一致。术后神经功能改善甚微。头颅筛查排除了任何颅内肿瘤。因此，诊断为原发性脊柱PNET。文献回顾显示，迄今为止仅报道了19例原发性脊髓内PNET病例，而本病例位于硬膜外。原发性脊髓内PNET是罕见肿瘤，预后较差。
