Can You Survive Ependymomas?

1. Patient age:
   • Infants have worse outcomes than children, and adults with tumors outside the spine have worse outcomes than children.
   • Infant treatment is tough, but the outcomes of intense chemotherapy have been quite positive.
   • Children younger than 19 years have a lower five-year survival rate of about 72 percent.
2. Tumor location: Infratentorial tumors have a poor prognosis. Spinal tumors have the best prognosis.
3. The extent of surgical resection:
   • Gross complete excision followed by targeted irradiation provides the highest probability of survival for grade II and III ependymomas.
   • Incomplete excision of grade II or III ependymoma has the poorest prognosis and may render the illness incurable.
   • If the illness does not advance, patients with full resection and subsequent radiation have a 5-year survival rate of 60 to 85 percent and a 10-year survival rate of 50 to 70 percent.
   • However, it is approximately 30 to 50 percent for incompletely resected ependymoma.
   • Children who had inadequate tumor removal after initial surgery or indications of tumor spread have a worse survival percentage.

It is not uncommon for an ependymoma to recur the following therapy, particularly in youngsters. If it returns, it is generally in the same location as the original tumor. There are ongoing clinical trials, testing new treatments and investigating how to reduce the long-term effects of treatments.

Ependymomas are a form of tumor that can develop in children and adults but are more prevalent in the juvenile population. It is a primary tumor that begins in either the brain or spine, both of which are parts of the central nervous system (CNS).

• Ependymoma tumors account for about 10 percent of all central nervous system (brain and spine) tumors in children.
• Depending on the aggressiveness of the tumor, an ependymoma can be classified as malignant (cancerous) or benign.
• They can arise anywhere in the brain or spine and have the potential to spread (disseminate or metastasize) to the spinal cord via the cerebrospinal fluid.

The symptoms of ependymoma depend on where the tumor is located, but may include the following 11 signs:

1. Nausea and vomiting
2. Lethargy and irritability
3. Headaches
4. Clumsiness
5. Difficulty with tasks like handwriting
6. Gradual decline in school performance
7. Changes in personality and behavior
8. Altered way of walking
9. Back pain
10. Difficulty walking
11. Problems with bowel and bladder control

The reasons for ependymoma formation are yet unclear but most studies have shown the following could be the possible causes or risk factors for the development of ependymoma:

1. Those who underwent brain radiation therapy as children had elevated chances of acquiring this tumor later in life.
2. Patients with neurofibromatosis type II (NF II) are more likely to develop spinal ependymoma.
3. Furthermore, it has been demonstrated that ependymoma is commonly connected with certain chromosomal abnormalities inside cells. Impaired cell growth and communication, as a result, could be contributing elements to the transition of a healthy cell into a cancer cell. The defects on chromosome 22 have been linked to the development of an ependymoma.
4. Approximately 90 percent of ependymomas in children develop in the skull, whereas the majority of ependymomas in adults form in the spine.
5. Ependymomas are uncommon later in life, accounting for just about two to three percent of adult primary brain tumors.
6. Ependymomas are often diagnosed at the age of four, but approximately one-third are diagnosed before the age of two years.

The diagnosis of ependymoma is usually done through many tests by eliminating other possible diseases or cancers. However, to confirm the diagnosis of ependymomas, doctors may recommend:

• Computed tomography scans
• Magnetic resonance imaging scans
• Lumbar puncture

Ependymomas are classed based on grade, histological features (how tumor cells appear under a microscope), and genetic traits.

Major types of ependymomas are:

• Subependymoma (grade I): A slow-growing ependymoma, which usually appears around a ventricle.
• Myxopapillary ependymoma (grade I): A kind of ependymoma that grows slowly and usually starts in the spinal column.
• Ependymoma (grade II): The most frequent kind, which usually develops inside or near the ventricular system. They are categorized into three subgroups based on histological features of the tumor cells:
  • Papillary ependymoma
  • Clear cell ependymoma
  • Tanycytic ependymoma
• Ependymoma, RELA fusion-positive (grades II to III):
  • An ependymoma that has been shown to have a particular fusion of the RELA and C11orf95 genes
  • Ependymomas with this specific genetic variation account for more than 70 percent of cerebrum ependymomas (the top portion of the brain above the cerebellum, also called supratentorial).
• Anaplastic ependymoma (grade III):
  • Rapidly developing ependymoma with a greater risk of recurrence.
  • Anaplastic ependymoma is most seen in the cerebellum or brainstem.

Ependymomas can be classified based on where they are in the brain.

• If an ependymoma is found in the cerebral hemispheres, it is called supratentorial ependymoma, which means “above the tentorium” (the membrane separating the top two-thirds of the brain from the bottom third).
• An ependymoma lying “below the tentorium” in the cerebellum or brainstem is called infratentorial ependymoma.

A precise course of therapy will be determined by the physician and surgeon depending on age, overall health and medical history, the kind, location, and size of the tumor, and the degree of the disease.

Treatment options for ependymoma include:

• Neurosurgery
  • The initial step to treat ependymomas is nearly always surgery.
  • Typically, surgery is performed initially to remove as much of the tumor as feasible.
  • Pediatric neurosurgeons could be unable to entirely remove a tumor due to its location or other circumstances.
  • Neuropathologists evaluate tumor tissues to determine the precise diagnosis. This information will be used by the child's doctor to plan the next stage of therapy.
• Endoscopic third ventriculostomy (ETV) or ventriculoperitoneal shunt (VP shunt)
  • When a tumor obstructs the flow of cerebral spinal fluid (CSF), surgeons may perform one of two operations to treat the symptoms of hydrocephalus or fluid buildup inside the skull.
  • Neurosurgeons perform an endoscopic third ventriculostomy to create a tiny opening that lets fluid pass around the obstruction. This surgery will be performed on about 90 percent of children who have hydrocephalus symptoms.
  • In certain situations, children could be treated with a VP shunt, where a tube from the ventricles to the stomach is inserted to drain excess fluid into the abdomen.
• Radiation
  • Tumors are damaged or shrunk by high-energy waves emitted by specialized equipment.
  • Radiation treatment is precisely targeted and dosed to eliminate cancer cells that remain after surgery.
  • This treatment could be administered to your child to limit the tumor's local growth.
  • If an ependymoma has spread, your child's doctor may advise them to get radiation to the entire brain and spinal cord.
• Chemotherapy
  • Chemotherapy medications inhibit the capacity of cancer cells to grow or reproduce, and they could be administered before surgery to decrease the tumor.
  • Chemotherapy medications function in several ways and are typically systemic therapies.
  • Chemotherapy could be administered orally, intramuscularly, intravenously, or intrathecally.

Therapy for ependymoma might vary depending on the location of the tumor and whether it is recurrent (does not disappear after initial treatment). Within the brain and posterior fossa, the greatest outcomes occur when the tumor is entirely removed surgically and high-dose focused radiation treatment is administered.

A similar method is utilized to treat spinal cord ependymomas. Depending on the child's age and an ependymoma that has spread or metastasized, more chemotherapy and radiation are administered.

Because ependymoma can arise in young children and therapy is administered during a critical period in the child's development. There may be some long-term repercussions of treatment, such as:

1. Growth and hormonal changes
2. Reduced fertility
3. Behavioral changes
4. Possible learning problems
5. Difficulties with coordination
6. Hearing and visual disturbances
7. Risk of developing second cancer later in life

All children treated for ependymoma needs long-term follow-ups. They could be examined at regular intervals to deal with any long-term repercussions of the treatment.