Intramedullary Spinal Cord Tumors in Children Homepage

Author

Rick Abbott, M.D.

Section Editors

Kristian Aquilina, M.D.

Editor in Chief

Rick Abbott, M.D.

Introduction

IMSCTs are rare tumors in children. They typically are benign and produce an extremely slow, insidious evolution of symptoms that depend on the location of the tumor. It is not uncommon for families to recognize the duration of symptoms only after the tumor has been discovered and they are asked to reflect upon aspects of their child’s motor development. The time span of this evolution is months to years. Untreated, IMSCTs will cause a steady but slow loss in motor function below the level of the tumor, and pain will become an important feature. Loss of sensation only develops late, and deterioration in bowel and bladder function is rare unless the tumor is in the conus of the spinal cord.

The most common type of IMSCT is the astrocytoma (40%) followed by gangliogliomas and other glial-neuronal tumors (35%), ependymomas (10-15%), hemangioblastomas and rarely other tumor.  Astrocytomas and gangliogliomas can be differentiated from ependymoma on MRI since the ependymoma arises centrally in the spinal cord (due to tumor growing from ependymal cells lining central canal) while the astrocytoma and gangliogliomas are eccentric positioned in the spinal cord.  The astrocytomas tend to be smaller (average length of 4 spinal segments) than gangliogliomas (average length of 8 spinal segments).  Hemangioblastomas intensely enhance and 50% will have cysts.

Currently, the preferred treatment for IMSCTs in children is surgical resection. Children with an 80+% resection have the same 5-year event-free survival as children treated with radiation but they benefit by avoiding the ill-effects of radiating a growing spine.  The use of intra-operative neurophysiologic monitoring of evoked motor potentials and, particularly, monitoring the D-wave potential in the motor pathways traveling through the levels of the spinal cord involved by the tumor has dramatically improved the safety margin for operating on. IMSCTs. Post-operative complications are seen in  8.9% of patients under the age of 18 who undergo resection of their IMSCTs with the frequency of complications increasing as the child becomes progressively more debilitated by the tumor.

Key Points

• Surgery same as radiation: The incidence of complications after surgery equals that seen when IMSCTs are treated with radiation.
• Timing of surgery: Treatment is better tolerated when a patient’s function is only minimally degraded. 
• Amount of resection: Radical resection is better tolerated than a biopsy.
• Management of recurrence: The risks of re-operation for tumor recurrence or progression are the same as those of a first-time operation and occur with a similar frequency.