Ependymoma

Ependymoma is divided into three grades: slowest-progressing types, most common intermediate type, and more aggressive fast-growing type. The most important factor determining course is how much of the tumor can be removed surgically; if nearly all tumor is removed, chances of long-term good outcome increase significantly, while residual tumor decreases this chance. Spinal ependymomas generally have better course than brain-located ones because complete removal is easier. Tumor's genetic subtype also affects its course.

Radiation therapy decision is based on tumor grade, how much was removed, and patient age. If lowest-grade tumor is completely removed, radiation therapy is usually not needed. If intermediate-grade tumor is completely removed, radiation therapy is debatable (some centers give it, others don't); if residual tumor remains, it is usually given. Radiation therapy is required for high-grade (aggressive) tumors. If tumor has disseminated to brain-spinal cord membranes, radiation therapy covering whole brain and spinal cord is applied. In very young children, radiation therapy is delayed if possible due to its effects on development.

Recurrence risk depends on how much tumor was removed. When tumor is completely removed and radiation therapy is given, recurrence risk is lower; when residual tumor remains, this risk increases. Ependymomas in the posterior fossa of the brain recur more frequently than those in the spinal cord and upper brain regions. Tumor's genetic subtype also affects recurrence risk. Most important step to reduce recurrence is complete surgical removal of tumor if possible, appropriate radiation therapy, and regular MRI follow-up.

Spinal cord ependymomas are usually located within the spinal cord, in the central canal. Part of the vertebral bone is opened to access the spinal cord, and under microscope, the tumor is carefully separated from spinal cord tissue and removed. The tumor usually has well-defined borders and can be largely completely removed in experienced hands. Surgery uses nerve monitoring (neuromonitoring) and tissue dissecting-aspirating devices. Possible risks include spinal cord injury and temporary neurological deterioration after surgery; most of these conditions improve with time. Most patients preserve or improve their function after surgery.

Role of drug therapy in ependymoma is limited; main treatments are surgery and radiation therapy. Drug therapy is used in following situations: in very young children to delay radiation therapy, when tumor recurs, and when dissemination to brain-spinal cord membranes occurs. New targeted therapies based on tumor genetic characteristics are still in research phase and are not standard treatment.

Ependymomas in the posterior fossa region of the brain extend toward the space through which brain-spinal fluid flows and obstruct fluid passage. When fluid cannot pass, it accumulates in brain cavities and hydrocephalus develops. Symptoms are severe headache, vomiting, vision problems, and altered consciousness. Rapid intervention is required; treatment options include opening a new fluid pathway endoscopically within the brain or redirecting fluid to another body region using a tube system (shunt). After tumor removal by surgery, fluid accumulation resolves in most patients.

In current classification, ependymomas are divided into genetic/molecular subtypes, which affect both prognosis and treatment decisions. One subtype of ependymoma in the posterior fossa region of the brain is seen in young children and has a worse prognosis, while another subtype is seen in adolescents and young adults and has a better prognosis. Certain genetic alterations in some tumors in the supratentorial region are associated with aggressive behavior, and treatments targeting these are being investigated. These tests are performed after surgery and guide treatment planning.

Yes, there are significant differences. In children, the tumor is more often located in the posterior fossa region of the brain, and the high-risk subtype seen at young ages may have a more aggressive course. In children, the side effects of radiation therapy (effects on learning and development, hormonal problems) are more prominent, so it is postponed if possible at very young ages, and proton therapy, which better preserves healthy tissue, may be preferred; chemotherapy can also be used to delay radiation therapy. In adults, the tumor is more often located in the spinal cord, generally has a better course, and the side effects of radiation therapy are less. Treatment is individually planned based on age, location, and genetic subtype.

Regular follow-up after treatment is very important because the tumor can recur. Generally, contrast-enhanced brain MRI is performed more frequently in the first years (every few months), and at longer intervals in subsequent years. If there is dissemination risk, spinal MRI is also added. On MRI, signs of recurrence may be a new mass or growth. In case of recurrence, options of re-operation, radiation therapy, or chemotherapy are considered; early detection increases treatment success. Follow-up also includes neurological examination, hormone tests in children who received radiation therapy, and developmental assessment when needed.

Preserving quality of life during and after treatment is important. Recommendations include: physical therapy and rehabilitation for balance and movement (especially after posterior fossa surgery); learning and memory support in children receiving radiation therapy; hormone tests and if needed hormone supplementation in those receiving radiation therapy in areas affecting hormonal balance; psychological support for patient and family; regular exercise and balanced nutrition; infection prevention education in patients with cerebrospinal fluid shunt; appropriate antiepileptic medication and safety precautions if seizures occur. Patients with spinal cord ependymoma can usually return to normal life. Pediatric patients should be followed up for long periods regarding growth and development.