Childhood Ependymoma Information -

By: peterhutch

Childhood ependymoma is a disease in which malignant (cancer) cells form in the tissues of the brain and spinal cord. The brain controls vital functions such as memory and learning, the senses (hearing, sight, smell, taste, and touch), and emotion. The spinal cord is made up of bundles of nerve fibers that connect the brain with nerves in most parts of the body. About 1 in 11 childhood brain tumors are ependymomas. Although cancer is rare in children, brain tumors are the most common type of childhood cancer other than leukemia and lymphoma.

The need for surgical intervention must be individualized on the basis of the extent of tumor, the length of time between initial treatment and the reappearance of the recurrent lesion, and the clinical picture. Patients with recurrent ependymomas who have not previously received radiation therapy and/or chemotherapy should be considered for treatment with these modalities.
In addition, patients may be candidates for focal retreatment with various radiation modalities, including stereotactic radiosurgery. Active agents include cyclophosphamide, cisplatin, carboplatin, lomustine, and etoposide.

Childhood ependymomas has been retrospectively investigated in total population samples from three hospitals in two cities. Regardless of the tumor's site of origin, the prognosis is grim. No child has been cured by surgery alone. The symptom-free interval after surgery is a first-order function of the age at diagnosis. The criteria for cure are best approximated by Collins "law," in terms of which there are apparently only three cured patients in this study.
Intracranial ependymomas are best treated by careful excision of the tumor and radiation of a generous area to a total dose of at least 4,500 rads over a 60-day period.

Ependymomas are tumors that arise from the cells lining the ventricles (the fluid containing spaces within the brain) and central canal within the spinal cord. According to the World Health Organization (WHO) classification of tumors, there are four types of ependymal tumors: myxopapillary ependymomas, subependymomas, ependymomas, and anaplastic ependymomas 5. The microscopic appearance and clinical behavior of these tumors vary considerably. Myxopapillary ependymomas are well-circumscribed tumors that occur primarily at the base of the spine in an area called the filum terminale. They are relatively uncommon in children. Subependymomas are well-circumscribed tumors that are usually located in or around the ventricles.

Children with primary brain tumors represent a major therapy challenge that,
for optimal results, requires the coordinated efforts of pediatric specialists in fields such as neurosurgery, neurology, rehabilitation, neuropathology, radiation oncology, medical oncology, neuroradiology, endocrinology, and psychology, who have special expertise in the care of patients with these diseases.[3-5] . More than one half of children diagnosed with brain tumors will survive 5 years from diagnosis. In some subgroups of patients, an even higher rate of survival and cure is possible.

After the childhood ependymoma has been removed, tests are done to find out if there is tumor remaining. The extent or spread of cancer is usually described as stages. For childhood ependymoma, tumors are described by grade and by where they are located in the central nervous system (brain and spinal cord). The grade of the tumor refers to how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. It is important to know the grade of the tumor and if there were any cancer cells remaining after surgery in order to plan treatment.

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