Joseph Junewick, MD FACR
over 7 years ago
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Joseph Junewick, MD FACR
|Diagnostic Category: Neoplasia Malignant
|Created: over 6 years ago
|Updated: over 6 years ago
9 year old female with 6 month history of intermittent headaches.
MR – Transmantle left cerebral neoplasm with cystic intraventricular component.
Ependymomas are glial tumors derived from differentiated ependymal cells lining the ventricles of the brain and the central canal of the spinal cord. They account for 3%–9% of all neuroepithelial neoplasms, 6%–12% of all pediatric brain tumors, and almost one-third of all brain tumors in patients younger than 3 years. Forty percent of ependymomas are supratentorial, while 60% are infratentorial in location. Ependymomas may manifest at any age with no gender predilection. The posterior fossa ependymoma arises most often in children (mean age, 6 years) whereas supratentorial ependymoma generally manifests in an older age group (mean age, 18–24 years).
Supratentorial ependymomas have a different clinical and radiologic presentation than infratentorial ependymomas. Patients with supratentorial ependymomas tend to present with focal neurologic deficits, headache, and seizures. The infratentorial ependymoma, due to its intraventricular location, will manifest secondary to increased intracranial pressure and hydrocephalus. The isocenter of supratentorial ependymoma is more commonly in the brain parenchyma. It is speculated that ependymomas may arise from embryonic rests of ependymal tissue trapped in the developing cerebral hemispheres. Supratentorial ependymomas tend to be larger in size than infratentorial ependymomas. Supratentorial lesions often contain a cystic component, while infratentorial lesion are often more solid tumors. Calcification is very common in both(40%–80% of cases).
The treatment of choice is total radical resection. Postoperative radiation therapy must be administered in every case of partially resected ependymomas as well as for those extraventricular ependymomas that are cystic or located near eloquent brain areas. The 5-year progression-free rate for children overall is about 50%. The 5- and 10-year survival rates for adults are 57.1% and 45%, respectively. Patients younger than 3 years have a significantly worse outcome than older children. In general, patients with supratentorial ependymomas have a better survival rate than patients with posterior fossa ependymomas.
Mermuys K, Jeuris W, Vanhoenacker PK, VanHoe L et al. Supratentorial Ependymoma. Radiographics (2005); 25:486-490.