Ependymoma Essentials - Episode 3: An Update on the ACNS0831 Clinical Trial (Feat. Drs. Smith, Foreman, Ellison and Tamiko Toland)

The purpose of this episode is to learn more about the status of the ACNS0831 trial, understand the significance of the trial to pediatric ependymoma research, and get clarity on how the results might impact the ependymoma community now and in the future.

D.Kovac, Neurora counsellor
Sina Falahati, MD, neurosurgical reviewer
Ependymomas are a rare type of gilal Tumors. They constitute 10 % of the Tumors occuring in the central nervous System. Males and females are equaly affected, especially children around five years of age, but also adults.
The ventricular system is a set of four interconnected cavities (ventricles) in the brain, where the cerebrospinal fluid celled CSF) is produced. Within each ventricle is a region of choroid plexus, a network of ependymal cells involved in the production of CSF. The ventricular system is continuous with the central canal of the spinal cord (from the fourth ventricle) allowing for the flow of CSF to circulate.
Risk factors are not clearly known. Neurofibromatosis type II has been associated with increased incidence with spinal chord ependymomas.
Most ependymomas are slow growing Tumors of children and Young adults, but there are also faster growing more malignant types. In the WHO classification of brain tumours ependymomas are divided into four Major subtypes - Myxopapillary ependymoma, subependymoma - both grade I, classic ependymoma - grade II and anaplastic ependymoma - grade III.
Depending on the Tumors location - posterior fossa lesions - lead to headache, Nausea, vomiting, Balance Problems called ataxia, dizziness and other Symptoms related to increased intracranial pressure. Sometimes seizurs or neurological deficits like muscle weakness are among the first Symptoms. Ependymomas within the spinal chord will usually Show Symptoms of muscle weakness, sensoric deficit and ataxia - all depending on the Tumors location and size.
All These Symptoms are not specific and can be related to many causes, many of them harmless.
Dissemination of the Tumor through the cerebrospinal fluid is not common, but is observed.
Radiologic Imaging - MRI and CT Scans can reveal the underlying cause of the Symptoms.
Usually MRI scan of the entire neuraxis - brain and spinal chord should be done to exclude metastases.
In most cases the initial Treatment of ependymomas should consist of maximal safe resection.
Chemotherapy Plays some roles in younger children or in some patients with bulk residual disease.
Incompletely resected ependymomas of grade II and grade III may require a short period of chemo-therapy and a second-look surgery.
Much in disease is still under research. It is recommended to visit specialized centers for the therapy of ependymomas.

KSNT News

Patients, caregivers and medical professionals from around the world dedicated May 3, 2020 as the ninth annual Ependymoma Awareness Day! cern-foundation.org.