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Ependymomas

The ependyma is the lining (epithelium) of the walls of the cavities (ventricles) inside the brain and the central canal of the spinal cord. Ependymomas are tumors that arise from this cell layer. Ependymal cells are related to the other supportive cells in the brain, which are called glial cells and include astrocytes and oligodendrocytes. The relatively rare ependymomas can occur either in the brain or in the spinal cord.

Ependymomas of the brain (intracranial) occur more often in children, whereas spinal ependymomas almost always occur in adults. The intracranial tumors usually are benign, clearly defined, and occur on the floor of the fourth ventricle of the brain. Ependymomas, regardless of their site, have the ability to spread to other parts of the nervous system through the cerebrospinal fluid, which surrounds the brain and spinal cord.

Symptoms

The symptoms of ependymomas are similar to those caused by other brain tumors and include headache, nausea, dizziness, and, less commonly, seizures. Depending on location, the tumors may cause other neurological symptoms, such as visual problems, neck pain, problems walking, changes in mood and personality, or speech difficulties.

Diagnosis

Imaging studies are the key component in the diagnosis of brain tumors. Currently, magnetic resonance imaging (MRI) is the best available imaging modality. Computed tomography (CT) scans also are used. For either study, an agent that provides contrast in the image is administered intravenously so neurological surgeons can visualize the tumor against the normal tissue in the background. In some cases, neurological surgeons may employ an MRI scan with frameless stereotactic guidance. For this study, a contrast MRI is performed after special markers (called fiducials) are placed on the patient's scalp. The fiducials are processed by a computer, which calculates the location of the tumor and creates a three-dimensional reconstruction. This image then is used at the time of surgery to help locate the tumor precisely, maximize tumor removal, and minimize injury to the surrounding brain.

Treatment

The first line of treatment for an ependymoma is surgical removal of the tumor without damage to the surrounding structures. The extent of the removal is a significant factor in the outcome. In addition, the surgical removal of the tumor will provide tissue samples, which are analyzed under a microscope for accurate diagnosis. After surgery, if there is evidence that the cancer has begun to spread in the cerebrospinal fluid, radiation therapy will be administered. Ependymomas are particularly sensitive to follow-up treatment with radiation. Stereotactic radiosurgery, which involves the use of a highly focused beam of radiation to target the cancer cells specifically and leave the surrounding brain unaffected, also may be used. Clinical studies have shown little benefit from chemotherapy, but new agents may have a role. The most common cause of death in patients with ependymomas is tumor recurrence or progression at the primary site.

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