Review of spinal neuroectodermal tumor.

ABSTRACT

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.