Primary intraspinal primitive neuroectodermal tumor (PNET): a rare occurrence

The concept of primitive neuroectodermal tumors (PNETs) has been evolving for many years, as has been its nomenclature. A 5 year old boy presented with pain in lower cervicodorsal region and left leg. Preoperative MRI of the spine and paravertebral region revealed a hyperintense lobulated lesion extending from D1-D4 with a large intraspinal and thoracic component. A total removal of tumor was achieved via a dorsal laminectomy and right posterolateral thoracotomy. The pathological findings were consistent with PNET. Post operative neurological examination had been unremarkable. Six months follow up scan showed no recurrence. A review of the literature shows that only 18 cases of primary intraspinal PNETs have been reported to date and the present case is exclusive, in which the tumor was thoracic, extradural in location and the child is alive at 8 months of follow up, with no evidence of tumor recurrence/metastasis. Primary intraspinal PNETs are rare tumors and carry a poor prognosis. Newer modalities of treatment should be tried to improve survival.