Primary spinal primitive neuroectodermal tumour: report of two cases mimicking neurofibroma and review of the literature

Abstract

Primary spinal primitive neuroectodermal tumours (PNETs) are a rare entity. Most of them occur in children and young adults. To date, 47 cases of primary spinal PNET have been reported in the literature. We present two cases of primary spinal PNET. In both cases, the tumours were thoracic extradural ones with intrathoracic extension through intervertebral foramina resembling neurofibroma. These tumours are highly aggressive with rapid growth as evidenced by the short history in both of our cases. Both cases underwent gross total removal of the intraspinal and thoracic components. Postoperatively, both patients underwent cranio-spinal radiotherapy. A review of the literature shows that the overall prognosis of PNETs of the spinal cord is very poor even with adequate surgery, radiotherapy and chemotherapy. One patient died after 4 months and the other one is still alive 8 months after surgery, radiotherapy and chemotherapy.