Brain and Spinal Cord Tumors

What are brain and spinal cord tumors?

The brain and spinal cord make up the central nervous system (CNS). CNS tumors may be benign (not cancer) or malignant (cancer). However, even benign tumors in the CNS can cause severe problems and even death.

Tumors that begin in the brain are called primary brain tumors. They may spread to other areas of the brain or spinal cord, but they do not usually spread to other parts of the body. Brain tumors may also come from tumor cells that have spread from other parts of the body. These are called metastatic or secondary brain tumors. These tumors are named for the part of the body where they begin and are rare in children.

Brain and spinal cord tumors are the 2nd most common type of cancer in children after leukemia. Approximately 20% of childhood cancers begin in the brain or spinal cord. There are about 4,100 new cases of CNS tumors in the United States each year. Brain and spinal cord tumors are the leading cause of pediatric cancer deaths.

Of pediatric brain tumors, about half are tumors called gliomas or astrocytomas. They form from glial cells, which make up the supportive tissue of the brain. Gliomas may be benign or malignant and can occur in different areas of the brain and the spinal cord.

The most common malignant brain tumor in children is medulloblastoma. This cancer forms in the cerebellum, in the back area of the brain in a region known as the posterior fossa.

The central nervous system (CNS)

The brain and spinal cord make up the central nervous system (CNS).  The central nervous system controls critical body functions including thinking, learning, emotions, speech, vision, movement, pain, breathing, and heart rate.

The brain has 3 main parts: the cerebrum, cerebellum, and brain stem.

• The cerebrum is the largest area of the brain.  The cerebrum is responsible for thinking, learning, speaking, memory, and emotions.
• The cerebellum is in the back area of the brain and controls motor function such as movement, balance, and coordination.
• The brain stem is the area that connects the brain and spinal cord.  It controls vital life functions such as breathing, heart rate, and blood pressure.

Messages travel back and forth from the brain to the rest of the body through the spinal cord and nerves.

Signs and symptoms of brain and spinal tumors

There are many types of tumors that form in the brain and spinal cord, and symptoms can vary widely.  Symptoms depend on where the tumor is located, the size of the tumor, how fast the tumor grows, and the child’s age and stage of development. 

Many childhood CNS tumors are slow growing.  The symptoms may be subtle, or they may come and go.  They might not follow a typical or predictable pattern.   

Some symptoms of brain tumors include:

• Headaches, especially in the morning* 
• Nausea and vomiting, especially in the morning*  

^{*Many childhood tumors are low grade. Typical presentation of headache and nausea/vomiting may not be seen.}

• Problems in vision
• Problems in speech, or hearing
• Loss of balance, coordination problems, or difficulty walking
• Weakness in the arms or legs
• Seizures
• Feeling tired or changes in activity level
• Unexplained weight change (gain or loss)
• Symptoms related to endocrine problems or changes in hormones such as increased thirst or early puberty
• Changes in personality
• Changes in school performance
• Problems with thinking or memory  
• Weakness, numbness, tingling, or changes in feeling on one side (arm or leg or both) of the body
• Chronic back pain (spinal cord tumors)
• Scoliosis (spinal cord tumors)
• Tilting the head to one side
• Increased head size in infants

Symptoms of spinal cord tumors include back or neck pain, weakness in arms or legs, difficulty walking, and problems going to the bathroom.

Diagnosis of brain and spinal tumors

Symptoms of brain and spinal cord tumors can be mild and hard to recognize at first. Early symptoms are often similar to other childhood illnesses. Depending on the type of tumor and how fast it grows, it can sometimes be a while before a tumor is diagnosed. Diagnosis usually includes a health history and physical exam, neurological exam, blood tests, and imaging tests.

• A health history and physical exam helps doctors learn about symptoms, general health, past illness, and risk factors.
• A neurological exam measures different aspects of brain function including memory, vision, hearing, muscle strength, balance, coordination, and reflexes.
• Imaging tests such as magnetic resonance imaging (MRI) and computerized tomography (CT) create detailed pictures of the brain and spinal cord. Doctors can see the size and location of the tumor and better understand what areas of the brain might be affected.
• Usually, doctors do a biopsy to diagnose the tumor. In a biopsy, a small sample of the tumor is removed during surgery. A pathologist looks at the tissue sample under a microscope to identify the specific type of tumor.
• A lumbar puncture may be performed to look for cancer cells in the spinal fluid.

Treatment of brain and spinal tumors

Surgery is a main treatment for tumors of the brain and spinal cord. Additional treatments for brain and spinal tumors include chemotherapy, radiation therapy, and targeted therapy.

Treatments depend on the type of tumor, where it is located, the age of the child, and how much of the tumor can be removed with surgery.

Most patients who have brain surgery will receive steroid medications to reduce swelling of the brain. Some patients may receive anti-seizure medications.

In some cases, a shunt is placed in the brain to prevent fluid from building up (hydrocephalus). A shunt is a small tube that drains cerebrospinal fluid so that it can be removed from the brain. The shunt may be temporary or permanent.

Post-surgery rehabilitation and supportive care including help with vision, speech, hearing, strength, and movement may facilitate recovery. Psychology and school support services can help with emotional, social, developmental, and learning needs.

Prognosis for brain and spinal tumors

The prognosis for brain and spinal cord tumors is based on many different factors. These include the type and grade of the tumor, molecular features of the tumor, age of the child, whether the tumor is new or has recurred, whether the cancer has spread to other areas, if the tumor can be removed with surgery, and how well it responds to treatments. The average survival rate for pediatric cancers of the brain and spinal cord is about 75%. But, the prognosis can be very different based on cancer type.

Life after brain and spinal tumors

The impact of a brain tumor on a child’s quality of life varies widely. Some children with brain tumors show minimal lasting effects on health and function after treatment. Other children may experience long-term problems in various areas of physical, cognitive, and emotional functioning.

Sometimes, problems are the result of injury to the brain from the tumor and/or surgery. Other changes may be due to long-term or late effects of chemotherapy and/or radiation.

Rehabilitation therapy and supportive care

Rehabilitation therapy can help address temporary or permanent problems in physical functioning after a brain tumor. Therapy may include physical therapy, occupational therapy, speech therapy, vision assistance, and hearing aids.

Patients treated for brain tumors should be monitored for changes or problems in functioning including:

• Physical function such as weakness, balance, coordination
• Cognitive function such as thinking, learning, memory, attention, processing information
• Behavior, emotions, and social function
• Speech, hearing, and vision problems
• Seizures
• Hormones and endocrine function

Psychological services can help with emotional, social, developmental, and cognitive needs. Additional support for return to school is often needed after treatment for a brain tumor. Neuropsychological assessment before and after treatment can help families identify education needs. The child’s care team can help parents plan for testing and academic accommodations.

Healthy behaviors

Simple lifestyle habits can help brain health and improve overall health.

• Eat healthy
• Be physically active
• Get enough sleep
• Manage stress

Late effects of therapy

For general health and disease prevention, all cancer survivors should adopt healthy lifestyle and eating habits, as well as continue to have regular physical checkups and screenings by a primary physician. Childhood cancer survivors treated with systemic chemotherapy and/or radiation should be monitored for acute and late effects of therapy.