Ependymoma

Childhood Cancer Name:

Ependymoma

What is Ependymoma?

Ependymoma is a primary tumor, which means that it starts in either the brain or spine. The brain and spine are part of the central nervous system (CNS).

Ependymomas arise from the cells lining the ventricles (the fluid containing spaces within the brain) and central canal within the spinal cord.

Characteristics:

Ependymomas are relatively rare tumors, accounting for 2–3% of all primary brain tumors. About 30% of pediatric ependymomas are diagnosed in children younger than three years of age. The cause is unknown.

Biology:

The most common types of cells in the central nervous system are neurons and glial cells. Tumors from neurons are rare. Glial cells are the cells that support the brain. Tumors that occur from these cells are called gliomas. Glial cell subtypes of the CNS include Astrocytes, Oligodendrocytes and Ependymal cells.

Main types of Ependymoma:

Ependymoma is a class of tumor that occurs in the brain or spinal cord. It includes different subtypes and grades. It is the third most common type of childhood brain tumor.

• Subependymomas (grade I): Typically slow-growing tumors.
• Myxopapillary ependymomas (grade I): Typically slow-growing tumors.
• Ependymomas (grade II): The most common of the ependymal tumors. This type can be further divided into the following subtypes, including cellular ependymomas, papillary ependymomas, clear cell ependymomas, and tancytic ependymomas.
• Anaplastic ependymomas (grade III): grows more rapidly and is more likely to spread to other parts of the brain and spine.

Most children have grade II or grade III variants occurring in the brain.

Childhood Cancer Localization:

Brain, spine.

• Anaplastic ependymomas occur most commonly Posterior fossa (back of the head).
• Myxopapillary ependymomas tend to occur in the lower part of the spinal column.
• Subependymomas usually occur near a ventricle.
• Ependymomas in children usually appear in the brain, most commonly in the fourth ventricle (or back part of the brain).

Childhood Cancer Noticeable Symptoms

Ependymomas appear in different locations within the brain and spinal column; symptoms depend on the location and size of the tumor. The biology may also be different based on the location.

• In babies, increased head size may be one of the first symptoms. Irritability, sleeplessness, unsteady gait (walking) and vomiting may develop as the tumor grows.
• In older children and adults, nausea, vomiting, and headache (mostly in the morning) are the most common symptoms.

Childhood Cancer Diagnostic method

CT scan (computed tomography), MRI (magnetic resonance imaging), biopsy, Lumbar puncture.

• CT scan or MRI is used in the initial evaluation of children suspected of having a tumor in the brain.
• Biopsy or surgical removal of tumor tissue for microscopic evaluation is necessary to make a definitive diagnosis.
• Evaluation of cerebrospinal fluid may be performed after surgery to detect tumor spread through the cerebrospinal fluid.
• Spine MRI is also usually performed.

Childhood Cancer Treatment

Surgery, radiation therapy (traditional and proton beam), chemotherapy.

• Surgery is the most important step of the treatment.  Surgery is the initial standard treatment for ependymoma, the neurosurgeon will attempt to remove as much of the tumor as possible without causing damage to the normal brain.
• Stereotactic radiosurgery – as opposed to whole brain irradiation – minimizes the dose of radiation delivered to healthy brain tissue and it can eradicate portions of the tumor not accessible through surgery.
• External beam radiation
• Conformal beam radiation therapy is a type of external beam radiation that contours the radiation beams to the shape of the tumor.
  Since ependymal tumors are relatively rare, the role of radiation and chemotherapy in the treatment of subependymomas and ependymomas is not clear enough.  Although chemotherapy is used extensively in children with ependymomas, there is little clinical evidence that chemotherapy improves survival of children with this type of tumor. Chemotherapy is reserved for patients with a residual tumor on scans after surgery.

• The majority of ependymal tumors do not respond to current chemotherapy regimens, but radiation therapy has been shown to be beneficial and is currently the standard-of-care for tumors that were not completely removed through surgery or tumors that have grown back after radiation therapy, or to delay radiation in infants and very young children.

• If the tumor has metastasized (spread) throughout the brain and spine systemic therapies (chemotherapy or radiation therapy). When a tumor cannot be safely resected and can be clearly diagnosed by imaging alone, a biopsy may offer no advantage in your child’s treatment plan, depending on the type of tumor. High-dose chemotherapy with autologous stem cell rescue can be used for some patients with recurrent brain tumors.
• Complete removal of the tumor is often not possible because of the tumor location and concerns about damaging the surrounding normal brain tissue.

Late effects of the treatment

Some treatments, while increasing survival, may have significant side effects on children. Prior to treatment, your child may undergo neuropsychological testing.

Follow up, late effects

MRI scans of the brain and/or spinal cord are usually done every three to four months for the first two years following diagnosis.

The main goals include

Preserve patient´s life.

Parents’ tips - From Parents of Children with Ependymoma

• You need to find out if the medical center where your child is diagnosed is an appropriate place for his or her treatment. The care of children with brain tumors requires multiple types of specialists (including neurosurgeons, oncologists, radiation oncologists, endocrinologists and many others).
• Do not hesitate to ask how many pediatric brain tumor patients they treat each year. Generally speaking, those that have the highest volume of childhood brain tumor patients also have greater knowledge of the disease, treatment options, and clinical trials.
• As parents, the best advice we can give to others with children battling Ependymoma is to discard all expectations you might have of your child, appreciate your child for who they are, take nothing for granted, and take heart from small victories.- Type of Cancer: Grade II Ependymoma, Diagnosed: Age 20 months, 2012 By Shannon (Ethan’s Dad) https://www.cern-foundation.org/awareness/story/making-the-most-of-every-day-with-ethan
• Cancer does not only affect the patient’s life but every single person in the child’s life. The brothers and sisters have to learn to live with part-time parents in and out of their lives as they arrange and re-arrange their schedules to keep up with everything. The siblings have to learn how to live without their brother or sister when they do go. – Type of Cancer: Ependymoma, Diagnosed: Age 4, 2007, By Kimberly (Ryan’s Mom).

Experts

• Find a center: http://www.cern-foundation.org/referrals
• For Stereotactic radiosurgery, Johns Hopkins Comprehensive Subependymoma and Ependymoma Research center: http://www.hopkinsmedicine.org/neurology_neurosurgery/research/labs/subependymoma_ependymoma_research/
• St. Jude is the only pediatric cancer research center that does not charge its patients´ families for treatment that is not covered by insurance. Thus, St. Jude has never refused to treat a child because of the family’s inability to pay. Learn more here: http://stjudedonate.weebly.com/faq.html

Testimonial

N/A at the moment

Hospitals/ Treatment Centers:

• Jude Children´s Research Hospital: https://www.stjude.org/disease/ependymoma.html
• American Brain Tumor Association: http://www.abta.org/brain-tumor-information/types-of-tumors/ependymoma.html?referrer=https://www.google.co.il/
• Johns Hopkins medicine: https://www.hopkinsmedicine.org/neurology_neurosurgery/research/labs/subependymoma_ependymoma_research/subependymoma_ependymoma.html

Links for more info

• Jude Children´s Research Hospital: https://www.stjude.org/disease/ependymoma.html
• American Brain Tumor Association: http://www.abta.org/brain-tumor-information/types-of-tumors/ependymoma.html?referrer=https://www.google.co.il/
• Answers to Questions You May Have When Your Child Is Diagnosed With a Brain Tumor http://www.curethekids.org/assets/documents/family-support/answers_web.pdf
• Ependymoma Guide, Collaborative Ependymoma Research Network: http://www.cern-foundation.org/uploads/docs/Ependymoma_Guide_2.pdf
• EpendyFamilies is a support group of families with a children whohas been diagnosed with ependymoma: https://www.facebook.com/groups/ependyparents/
• Fighting Ependymoma: http://www.fightingependymoma.org.uk/index.php/how-to-help
• The Childhood Brain Tumor Foundation http://www.childhoodbraintumor.org/medical-information/brain-tumor-types-and-imaging/item/84-ependymomas
• The Cern foundation- collaborative Ependymoma Research Network; https://www.cern-foundation.org/education/resources/ependymoma-resources

Reseacrh

To understand how brain tumors develop in children and identify personalized treatment strategies visit ‘The Pacific Pediatric Neuro-Oncology Consortium (PNOC)’-  http://www.pnoc.us/about