Patnaik Ashis, Mishra Sudhansu S, Mishra Sanjib, Deo Rama C
Department of Neurosurgery, SCB Medical College & Hospital, Cuttack, Odisha, India.
Br J Neurosurg. 2013 Feb;27(1):2-6. doi: 10.3109/02688697.2012.724120. Epub 2012 Sep 14.
Primary spinal primitive neuroectodermal tumors (PNETs) are rare tumors. Most of these tumors occur in children and young adults. To date, 47 cases of primary spinal PNET have been reported in the literature. These tumors are highly aggressive with rapid growth. Review of the literature shows that the overall prognosis of PNETs of spinal cord is very poor even with adequate surgery, radiotherapy and chemotherapy. All the cases reported to date are reviewed in terms of surgical treatment, adjuvant therapy and outcome and the experience with two of these cases are described. Both cases were thoracic extradural ones with intrathoracic extension through intervertebral foramina resembling neurofibroma. Both cases underwent gross total removal of intraspinal and thoracic component. Post-operatively both underwent cranio-spinal radiotherapy. One patient died after a post-operative period of 4 months and the other one is still alive 8 months after surgery, radiotherapy and chemotherapy.
原发性脊柱原始神经外胚层肿瘤（PNETs）是罕见肿瘤。这些肿瘤大多发生于儿童和年轻人。迄今为止，文献中已报道47例原发性脊柱PNET。这些肿瘤具有高度侵袭性，生长迅速。文献回顾显示，即使进行了充分的手术、放疗和化疗，脊髓PNET的总体预后仍很差。对迄今报道的所有病例进行了手术治疗、辅助治疗及预后方面的回顾，并描述了其中两例的治疗经验。两例均为胸段硬膜外肿瘤，经椎间孔向胸腔内延伸，类似神经纤维瘤。两例均行脊髓内及胸腔内肿瘤全切术。术后均接受全脑全脊髓放疗。1例患者术后4个月死亡，另1例在手术、放疗及化疗后8个月仍存活。
