Pediatric Brain Tumors
After discovering your child has a brain tumor, one of the first things you can do is learn more about his or her unique situation. The information and resources in this guide will get you started. The more you know, the more prepared and comfortable you will be moving forward.
Brain and spinal cord tumors are the second most common type of childhood cancer. Although their cause is unknown, doctors know they occur when abnormal cells form in the tissues of the brain or spinal cord. They may be low grade or high grade. Both types may grow and press on nearby areas of the brain, but low-grade tumors rarely spread. Both, however, typically need treatment.
The brain and spinal cord make up the central nervous system (CNS). When a tumor grows into or presses on an area of the brain or spine, it may stop that part of the brain or spine from working the way it should. The brain is made up of the cerebrum, cerebellum and brain stem. Each controls critical functions in the body (see Overview).
The signs and symptoms of childhood brain and spinal cord tumors differ in every child, depending on the following:
- Where the tumor arises — in the brain or spinal cord
- The size of the tumor
- How fast the tumor grows
- The child’s age and development
Diagnosing and Treating a Brain Tumor
A variety of tests and procedures may be used to diagnose brain and spinal cord tumors (see Diagnosing and Biomarkers). A biopsy may be done to remove a sample of tissue. Most childhood brain tumors are diagnosed conclusively through a biopsy or resection. The biopsy is often done through a small needle while a resection is achieved by removing part of the skull to identify and remove some or all of the tissue.
A pathologist views the tissue under a microscope to identify the type of cancer cells. This helps guide the neurosurgeon in the type of resection needed. The grade of the tumor is based on how abnormal the cancer cells look under a microscope and how many of the tumor cells are actively dividing.
After a diagnosis is made, finding a doctor or treatment facility that specializes in treating your child’s type of brain tumor is important because treating children with brain tumors is not just treating cancer in smaller adults. Children don’t have the same mutations that cause adult brain tumors, and they don’t respond to treatment the same way.
Your child’s medical team will likely include a variety of specialists, such as medical oncologists, radiation oncologists, neurologists, neurosurgeons, pathologists, rehabilitation specialists and more. They are specially trained to work with children, which will be evident in their medical expertise and their bedside manner. They will work closely with you on the treatment plan they feel is best for your child’s unique situation after considering the type of tumor, your child’s age and overall health, how fast the tumor is growing, where the tumor has formed and if it has spread to nearby tissue or to other parts of the body.
You may find comfort in the fact that advances surrounding pediatric brain tumor treatment are offering a great deal of promise. In the last five years, researchers have discovered changes in genes, chromosomes and proteins inside brain tumor cells that can be used to help predict a child’s prognosis (outlook) and help guide treatment.
Other advances include new and refined surgical techniques that have made surgeries safer and more successful; innovative ways for chemotherapy drugs to cross the blood-brain barrier (the network of blood vessels and tissue that protects the brain from harmful substances); targeted therapy drugs to inhibit the gene changes that help tumors grow; and vaccines to stimulate the body’s immune cells to recognize and destroy brain tumor cells. Additionally, multiple clinical trials on other areas of research are underway.
As you discuss treatment options with your medical team, ask about potential side effects and late effects, which are side effects that can occur months or even years after treatment ends.
Common Pediatric Brain Tumors
Below is information on many types of brain tumors diagnosed in children.
Childhood Central Nervous System Embryonal Tumors
Central nervous system (CNS) embryonal tumors may begin in embryonic (fetal) cells that remain in the brain after birth. They tend to spread through the cerebrospinal fluid (CSF) to other parts of the brain and spinal cord. There are different types of CNS embryonal tumors.
These fast-growing tumors that form in brain cells of the cerebellum are the most common CNS embryonal tumors. These tumors can spread through the spinal fluid to other parts of the brain and spine. In rare cases, medulloblastomas can spread to the bone, bone marrow, lung or other parts of the body.
Non-medulloblastoma embryonal tumors
Non-medulloblastoma embryonal tumors are fast-growing tumors that usually form in brain cells outside of the cerebellum in the cerebrum. They may also form in the brain stem or spinal cord. There are four types of these tumors.
Embryonal tumors with multilayered rosettes (ETMR) are rare tumors that form in the brain and spinal cord. ETMRs most commonly occur in young children and are fast growing.
Medulloepitheliomas are fast-growing tumors that usually form in the brain, spinal cord or nerves just outside the spinal column. They occur most often in infants and young children.
CNS neuroblastomas are very rare tumors that form in the nerve tissue of the cerebrum or the layers of tissue that cover the brain and spinal cord. CNS neuroblastomas may be large and spread to other parts of the brain or spinal cord.
CNS ganglioneuroblastomas are rare tumors that form in the nerve tissue of the brain and spinal cord. They may form in one area and be fast growing, or form in more than one area and be slow growing.
CNS embryonal tumors, including medulloblastomas, are typically treated with surgery, radiation therapy and/or chemotherapy. A type of immunotherapy may be used to treat a certain form of high-risk neuroblastomas. Because of the damage radiation therapy can cause to the brains of infants and young children, radiation therapy may not be used.
Glioma is a general term for any cancer that develops from the glial cells. There are multiple types of glial cells: astrocytes, oligodendrocytes and ependymal cells. Glial cells hold nerve cells in place and help protect them. Following are some of the more common types of gliomas that affect children.
Astrocytomas are tumors that begin in star-shaped brain cells called astrocytes. They are the most common type of glioma diagnosed in children and can form anywhere in the central nervous system (brain, brain stem and spinal cord).
An astrocytoma is typically treated with surgery and, if low-grade, observation. Radiation therapy, with or without chemotherapy, is usually needed for high-grade tumors. If necessary, a cerebrospinal fluid diversion procedure may also be required. As a result of the discovery of specific mutations in some pediatric astrocytomas, the use of targeted therapy is now being tested.
Ependymomas form from ependymal cells that line the ventricles and passageways in the brain and the spinal cord. The part of the brain that is affected depends on where the ependymomas develop.
Surgery and radiation therapy are standard treatments for these tumors. Chemotherapy may be used if the surgeon cannot remove all of the tumor at the time of diagnosis.
Brain Stem Gliomas
Childhood brain stem gliomas are most often one of two types.
Diffuse intrinsic pontine gliomas (DIPGs) are high-grade tumors of the pons. The pons is located in the middle part of the brain stem and is the primary control center for many critical functions, such as breathing, heartbeat, alertness (wakefulness), control of movement of the eyes and mouth and swallowing. DIPGs are fast growing and spread aggressively by invading adjacent areas of the brain and spine. They are hard to treat because of their location.
Focal gliomas are slow growing and can be found in multiple areas of the brain stem. These are usually low grade and are very responsive to therapy.
Surgery, radiation therapy, chemotherapy, cerebrospinal fluid diversion, observation and/or targeted therapy are the standard treatments for these brain tumors.
Central Nervous System Germ Cell Tumors
CNS germ cell tumors form in the brain or spinal cord from germ cells, a type of cell present in an unborn baby. CNS germ cell tumors commonly form near the pineal gland and/or in an area of the brain that includes the pituitary gland and the tissue just above it. Three types of treatment are used, including surgery, radiation therapy and chemotherapy.
Germinomas are the most common type of childhood CNS germ cell tumors. One type of germinoma is an intracranial germinoma, which is also known as a dysgerminoma or an extra-gonadal seminoma.
Nongerminomas are less common and often make certain proteins or hormonal markers. Types include embryonal cell carcinomas, choriocarcinomas, yolk sac tumors, teratomas and mixed germ cell tumors.
These rare, benign brain tumors are found near the pituitary gland. They are usually part solid mass and part fluid-filled cyst. They are noncancerous and do not spread to other parts of the brain or to other parts of the body. They can, however, cause permanent damage to the parts of the brain where they arise. As they grow and press on nearby parts of the brain or other areas, they may affect hormone production (too much or too little), growth and vision. Treatments include surgery (resection), followed by focal radiation therapy in some patients.
Choroid Plexus Tumors
The choroid plexus is a network of blood vessels and cells in the ventricles (fluid-filled spaces) of the brain that make cerebrospinal fluid. These rare tumors can occur in children and adults, but are more common in children. When these tumors form, they can block cerebrospinal fluid pathways in the brain, grow and compress important brain structures, and/or overproduce cerebrospinal fluid.
The majority of choroid plexus tumors are benign (non-cancerous) but can sometimes become malignant (cancerous). Choroid plexus papillomas are Grade I and mostly benign. Atypical choroid plexus papillomas are Grade II. Choroid plexus carcinomas are Grade III, the rarest type of choroid plexus tumor, and are malignant.
|Other Types of Brain Tumors Found in Children and Adults*
|Diffuse astrocytic and oligodendroglial tumors
Giant cell glioblastoma
Diffuse midline glioma
|Other astrocytic tumors
Anaplastic pleomorphic xanthoastrocytoma
Clear cell ependymoma
|Neuronal and mixed neuronal-glial tumors
Embryonal tumor with multilayered rosettes
CNS embryonal tumor
Atypical teratoid/rhabdoid tumor
CNS embryonal tumor with rhabdoid features
|Tumors of the cranial and paraspinal nerves
|MPNST with perineural differentiation
|Mesenchymal, nonmeningothelial tumors
Solitary fibrous tumor/hemangiopericytoma
Ewing sarcoma/peripheral primitive neuroectodermal tumor
Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma
|Anaplastic large cell lymphoma
Langerhans cell histiocytosis
Pediatric Supportive Care
Parents instinctively feel they should be able to fix what ails their child, so it’s understandable that you may feel helpless right now. You’re not. Although this experience is overwhelming, helping your child – and your family – adapt to daily life during and after treatment is vital. Accept help when it’s offered, and trust your instincts.
Maintain the household routine. Stick to the family schedule as much as you can. Kids – and adults – need to feel some sense of normalcy during a time of family stress. That means school activities, social events and family dinners must go on, as much as possible. Accept offers of help for errands, meals and carpooling from neighbors, family members or friends. At home, ask everyone to do their part to keep the household running.
Include siblings in the conversation. Telling your kids that their sibling has cancer may be difficult, but you know better than anyone how they may react and how best to support them. The basic information all kids will likely need is the name of the cancer, the body part it affects, how it will be treated and how their lives will be affected. Use age-appropriate language. Younger children may only understand that their brother or sister is sick and needs medicine to get better, whereas older children will likely want more information. Siblings can even help with caregiving. Just keep the responsibilities age appropriate. Keep in mind that teens still need to be teens. Although they may be very capable of picking up more slack, time to hang out with their friends is essential. Your child’s siblings may feel a variety of emotions, from fear and guilt to anger. Encourage them to share their feelings and ask questions. It’s important to address their concerns. Set aside time for each child and involve them in caregiving, as appropriate.
Understand cognitive issues. Your child may have difficulties with thinking, remembering and other cognitive skills. This could make it more difficult to concentrate on any number of tasks. In some cases, it may result in a learning disability. If that is the case, your medical team will talk with you about the best way to manage it. Children’s brains are still developing, and it may be possible to train certain parts of the brain to do things they normally wouldn’t do.
Manage emotional issues. Talk with a therapist on your child’s medical team about how to address the changes in your child’s mood and behavior and what to do if new symptoms arise. Additionally, your child – and you – may fear the possibility of recurrence. This feeling may cause anxiety and stress. Consider yoga or other relaxation techniques. Your child’s therapist can recommend additional coping strategies.
Prepare for returning to school. Your child’s doctor will determine if returning to school is medically appropriate for your child. If so, it’s a good idea to work with the doctor and the school to determine the best course of action. It may help to have a member of the medical team contact the school to explain necessary precautions, make a plan for managing side effects and answer any questions they may have. Cognitive and speech impairment issues may make school work more challenging. Talk with your child’s school about developing an individualized education plan (IEP) to address challenges and how to overcome them. Consider getting a tutor to keep your child from falling behind. Keep in mind that although your child may be excited to return to school, he or she might also be afraid of how classmates will react. Sometimes kids don’t know how to act around someone who is or has been ill. Make a plan for how your child can respond to or ignore those instances.
Learn about late effects. Also referred to as long-term side effects, late effects can begin weeks, months or even years after treatment. Ask your medical team about what to expect and what to do if and when one begins.
Make a comprehensive follow-up plan. Children who are treated for any type of cancer should have a long-term, even lifelong, plan for checkups to monitor for recurrence (cancer that comes back). Hearing and vision issues are common after treatment for a brain tumor, making regular testing necessary. Regular dental checkups become more important after cancer treatment because of an increased risk for cavities, thinning of tooth enamel and problems with the roots of teeth. Make sure your child’s current and future doctors and dentists are informed about your child’s medical history.
Monitor hormonal status. Children should be monitored closely during puberty and afterward to check for growth issues and reproductive issues. Especially if your child is very young, you may think fertility issues aren’t a concern. However, it’s important to have an endocrinologist be involved in your child’s ongoing care as fertility issues may arise as a result of the tumor or its treatment.
Rely on the resources available. Your child’s medical team is specially trained to guide you and your child through treatment and rehabilitation to follow-up care and emotional support. Ask for their help. Pediatric brain tumors offer a unique set of challenges, and you will be better equipped to manage them if you have a solid support system.
Take care of the caregiver. This means you. Keep up with your medical appointments. Exercise daily, even if it’s just for 10 minutes. Relax and think about something completely unrelated to cancer. Join a support group for parents of children with brain tumors. Don’t feel guilty about focusing on yourself. You must tend to your own physical and emotional needs to be your best self for your child.
Strive for Routine + Flexibility
Keeping daily life as normal as possible for your child is important. Although change can be difficult, try being open to doing things differently. Lean on family and friends. Find a support group. Advocacy organizations can put you in touch with parents of other pediatric brain tumor patients who can be a wealth of help because they know just what you’re going through. The following suggestions may help you manage the unique challenges that come with caring for a child with a brain tumor:
- Get creative with food. Traditional meals and mealtimes may go by the wayside for the time being, and that’s OK. Changes in appetite are common. Instead of stressing about it, let your child eat whenever he or she feels hungry. Offer high- or low-calorie snacks, as appropriate, throughout the day. Make mealtimes fun by serving food in different shapes on brightly colored plates. Spread a blanket on the family room floor for a family picnic.
- Help your child stay connected. Arrange playdates for a younger child and encourage your older child to invite friends over to watch a movie. Phone calls, texts, video chats and social media platforms make it easier for your child to stay in touch with friends, especially when an in-person visit may not be possible.
- Explore new hobbies. Keep your child entertained with activities that can be done during hospital stays or at home when visitors aren’t recommended. If you notice your child is frequently sad or appears to be depressed, call the doctor.
- Be positive. Show your child that you’re optimistic about the future. Children typically take cues from their parents’ behavior. Your upbeat attitude may rub off. If you’re having a rough time, talk with your child’s medical team about support groups, a therapist or other resources.