Ependymoma
The Common Vein Copyright 2010
Definition
An ependymoma is a tumor that is derived from ependymal cells, which line the ventricular surface.
It usually arises in children, though in can occur in adults, in whom it occurs in the spinal cord, being more commonly located in the lumbosacral spinal cord and filum terminale. The latter is usually referred as a myxopapillary ependymoma.
Morphologically, the tumor cells may form gland-like structures that resemble the embryologic ependymal canal, with processes extending into the lumen.
Functionally, because the tumors usually arise in the spinal cord, a progressive myelopathy develops, resulting in weakness of the extremities, spasticity of movement and altered/diminished sensation. If the tumor in intracranial, then, as in all intracranial tumors, headaches, nausea & vomiting, ataxia, dizziness, focal and brainstem symptoms can develop. A cauda equina syndrome can also be part of the presentation.
When it occurs in children younger than 2, then the prognosis tends to be poor. Treatment is usually with surgical resection and radiotherapy, where the rate success is significantly good (above 70%). Chemotherapy itself does not usually have a role in treatment, as it has not been shown to be effective.
Radiologically, MRI is the preferred modality. It demonstrates the tumor as a well-demarcated lesion, being heterogenous with calcifications. It sohes contrast enhancement. Hemorrhage can also be seen.