Brain Tumors

What is a brain tumor?

Brain tumors are the most common solid tumors in children. Approximately 4,000 children and adolescents in the U.S. are diagnosed with primary brain tumors each year. Primary brain tumors start in the brain and generally do not spread outside the brain tissue. Most central nervous system cancers are brain tumors. Brain tumors, either malignant or benign, are tumors that originate in the cells of the brain. A tumor is an abnormal growth of tissue.

A benign tumor does not contain cancer cells and usually, once removed, does not recur. Most benign brain tumors have clear borders, meaning they do not invade surrounding tissue. These tumors can, however, cause symptoms similar to cancerous tumors because of their size and location in the brain.

Malignant brain tumors contain cancer cells. Malignant brain tumors are usually fast growing and invade surrounding tissue. Malignant brain tumors very rarely spread to other areas of the body, but may recur after treatment. Sometimes, brain tumors that are not cancer are called malignant because of their size and location, and the damage they can do to vital functions of the brain.

Brain tumors can occur at any age. Brain tumors that occur in infants and children are very different from adult brain tumors, both in terms of the type of cells and the responsiveness to treatment.

The American Cancer Society states that no apparent reason can be found for the development of brain tumors in children. Brain tumors cannot be directly linked to something the parents or child did or did not do. 

In addition, both the American Academy of Pediatrics and the National Cancer Institute recommend that children receive cancer diagnosis and treatment at one of the more than 200 medical centers in the U.S. that specialize in pediatric cancers. The organizations cite research studies that show 20% to 40% higher survival rates when children receive care in such specialized centers. 

Anatomy of the brain

The central nervous system (CNS) consists of the brain and spinal cord. The brain is an important organ that controls thought, memory, emotion, touch, motor skills, vision, respirations, temperature, hunger, and every process that regulates our body.

The brain can be divided into the cerebrum, the brainstem, and the cerebellum:

 Anatomy of the brain, child


  • Cerebrum (front of brain). The cerebrum is composed of the right and left hemispheres. Functions of the cerebrum include: initiation of movement, temperature sensation, touch, vision, hearing, judgment, reasoning, problem solving, emotions, and learning.
  • Brainstem (base of brain). This includes the midbrain, the pons, and the medulla. Functions of this area include: movement of the eyes and mouth, relaying sensory messages (for example, hot, pain, or loud), hunger, respirations, consciousness, cardiac function, body temperature, involuntary muscle movements, sneezing, coughing, vomiting, and swallowing.
  • Cerebellum (back of brain). Located at the back of the head, the cerebellum's function is to coordinate voluntary muscle movements and to maintain posture, balance, and equilibrium.
Prevention & Risk Treatment

Prevention & Risk Treatment

What causes brain tumors?

The majority of brain tumors have abnormalities of genes involved in cell cycle control, causing uncontrolled cell growth. These abnormalities are caused by alterations directly in the genes, or by chromosome rearrangements that change the function of a gene.

Patients with certain genetic conditions (neurofibromatosis, von Hippel-Lindau disease, Li-Fraumeni syndrome, and retinoblastoma) also have an increased risk for developing tumors of the central nervous system. There have also been some reports of children in the same family developing brain tumors who do not have any of these genetic syndromes.

Some chemicals may change the structure of a gene that protects the body from diseases and cancer. Research has been investigating parents of children with brain tumors and their past exposure to certain chemicals, including pesticides and petroleum products.

Children who have received radiation therapy to the head as part of prior treatment for other malignancies are also at an increased risk for new brain tumors.

What are the symptoms of a brain tumor?

The following are the most common symptoms of a brain tumor. However, each child may experience symptoms differently. Symptoms vary depending on size and location of tumor. Many symptoms are related to an increase in pressure in or around the brain, except in very young children (whose skull bones have not yet fused together), as there is no spare space in the skull for anything except the delicate tissues of the brain and its fluid. Any tumor, extra tissue, or fluid can cause pressure on the brain and result in the following symptoms:

  • Increased intracranial pressure (ICP). ICP is caused by extra tissue or fluid in the brain. Pressure may increase because one or more of the ventricles that drain cerebrospinal fluid (CSF, the fluid that surrounds the brain and spinal cord) has been blocked, causing the fluid to be trapped in the brain. Increased ICP can cause the following:

    • Headache

    • Vomiting (usually in the morning)

    • Nausea

    • Personality changes

    • Irritability

    • Drowsiness

    • Depression

    • Decreased cardiac and respiratory function and eventually coma if not treated

  • Symptoms of brain tumors in the cerebrum (front of brain) may include:

    • Seizures

    • Visual changes

    • Slurred speech

    • Paralysis or weakness on half of the body or face

    • Increased intracranial pressure (ICP)

    • Drowsiness and/or confusion

    • Personality changes/impaired judgment

    • Short-term memory loss

    • Gait disturbances

    • Communication problems

  • Symptoms of brain tumors in the brainstem (base of brain) may include:

    • Seizures

    • Endocrine problems (diabetes and/or hormone regulation)

    • Visual changes or double vision

    • Headaches

    • Paralysis of nerves/muscles of the face, or half of the body

    • Respiratory changes

    • Increased intracranial pressure (ICP)

    • Clumsy, uncoordinated walk

    • Hearing loss

    • Personality changes

  • Symptoms of brain tumors in the cerebellum (back of brain) may include:

    • Increased intracranial pressure (ICP)

    • Vomiting (usually occurs in the morning without nausea)

    • Headache

    • Uncoordinated muscle movements

    • Problems walking (ataxia)

The symptoms of a brain tumor may resemble other conditions or medical problems. Some pituitary tumors may cause diabetes insipidus, with symptoms of abnormal electrolytes and changes in mental status. Some people with deep thalamic tumors have abnormal eating patterns. These tumors may be particularly difficult to diagnose in adolescents because of the symptoms. Always consult your child's doctor for a diagnosis.

Diagnosis

Diagnosis

How are germ cell tumors diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for germ cell tumors may include:

  • Biopsy. A sample of tissue is removed from the tumor and examined under a microscope.

  • Complete blood count (CBC). This measures size, number, and maturity of different blood cells in a specific volume of blood.

  • Additional blood tests. These tests may include blood chemistries, evaluation of liver and kidney functions, tumor cell markers, and genetic studies.

  • Multiple imaging studies, including:

    • Computed tomography (CT) scan. This is a diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce horizontal, or axial, images (often called slices) of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general X-rays.

    • Magnetic resonance imaging (MRI). This is a diagnostic procedure that uses a combination of large magnets, radio frequencies, and a computer to produce detailed images of organs and structures within the body, without the use of X-rays.

    • X-ray. This diagnostic test uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.

    • Ultrasound (also called sonography). This is a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.

    • Bone scans. This involves pictures or X-rays taken of the bone after a dye has been injected that's absorbed by bone tissue. These are used to detect tumors and bone abnormalities.

What are the different types of germ cells?

Diagnosis of germ cell tumors depends on the types of cells involved. The most common types of germ cell tumors include:

  • Teratomas. Teratomas contain cells from the three germ layers: ectoderm, mesoderm, and endoderm. Teratomas can be malignant or benign, depending on the maturity and other types of cells that may be involved. Teratomas are the most common germ cell tumor found in the ovaries. Sacrococcygeal (tail bone, or distal end of spinal column) teratomas are the most common germ cell tumors found in childhood. Because these sacrococcygeal tumors are often visible from the outside of the body, diagnosis is made early and treatment and/or surgery are initiated early, making the prognosis for this type of germ cell tumor very favorable.

  • Germinomas. Germinomas are malignant germ cell tumors. Germinomas are also termed dysgerminoma when located in the ovaries; and seminoma when located in the testes. Among children, germinoma, or dysgerminoma, occurs most frequently in the ovary of a prepubescent or adolescent female. Dysgerminoma is the most common malignant ovarian germ cell tumor seen in children and adolescents.

  • Endodermal sinus tumor or yolk sac tumors. Endodermal sinus tumor or yolk sac tumors are germ cell tumors that are most often malignant, but may also be benign. These tumors are most commonly found in the ovary, testes, and sacrococcygeal areas (tail bone, or distal end of spinal column). When found in the ovaries and testes, they're often very aggressive, malignant, and can spread rapidly through the lymphatic system and other organs in the body. Most yolk sac tumors will require surgery and chemotherapy, regardless of stage or presence of metastasis, because of the aggressive nature and recurrence of the disease.

  • Choriocarcinoma. Choriocarcinoma is a very rare, but often malignant germ cell tumor that arises from the cells in the chorion layer of the placenta (during pregnancy, a blood-rich structure through which the fetus takes in oxygen, food, and other substances while getting rid of waste products). These cells may form a tumor in the placental cells during pregnancy and spread (metastasize) to the infant and mother. When the tumor develops during pregnancy, it's called gestational choriocarcinoma. Gestational choriocarcinoma most often occurs in pregnant females who are between ages 15 and 19. If a nonpregnant young child develops choriocarcinoma from the chorion cells that originated from the placenta that are still in the body, the term used is nongestational choriocarcinoma.

  • Embryonal carcinoma. Embryonal carcinoma cells are malignant cells that are usually mixed with other types of germ cell tumors. They occur most often in the testes. These types of cells have the ability to rapidly spread to other parts of the body. When these cells are mixed with an otherwise benign type of tumor (mature teratoma), the presence of embryonal carcinoma cells will cause it to become malignant (cancerous).

Many germ cell tumors have multiple types of cells involved. The diagnosis, treatment, and prognosis are based on the most malignant of the cells present and the majority type of cells that are present.

Treatments

Treatments

Treatment for brain tumors

If your child has been diagnosed with a brain tumor, you may want to consider getting a second opinion before beginning treatment. In fact, some insurance companies require a second opinion for such diagnoses. According to the American Cancer Society, it is very rare that the time it will take to get a second opinion will have a negative impact on your treatment. The peace of mind a second opinion provides may be well worth the effort. 

Specific treatment for brain tumors will be determined by your child's doctor based on:

  • Your child's age, overall health, and medical history

  • Type, location, and size of the tumor

  • Extent of the disease

  • Your child's tolerance for specific medications, procedures, or therapies

  • Expectations for the course of the disease

  • Your opinion or preference

Treatment may include (alone or in combination):

  • Surgery. Surgery is usually the first step in the treatment of brain tumors. The goal is to remove as much of the tumor as possible while maintaining neurological function. Surgery for a biopsy may also be done to examine the types of cells the tumor is made of for a diagnosis. This is frequently done if the tumor is in an area with sensitive structures around it that may be injured during removal.

  • Chemotherapy

  • Radiation therapy

  • Steroids (to treat and prevent swelling especially in the brain)

  • Antiseizure medication (to treat and prevent seizures associated with intracranial pressure)

  • Ventriculoperitoneal shunt (also called a VP shunt). A VP shunt may be placed in the head to drain excess fluid from inside the brain to the abdomen. A VP shunt helps control the pressure inside the brain.

Illustration demonstrating ventriculoperitoneal shunt placement
  • Bone marrow transplantation

  • Supportive care (for the side effects of the tumor or treatment)

  • Rehabilitation (to regain lost motor skills and muscle strength; speech, physical, and occupational therapists may be involved in the health care team)

  • Antibiotics (to treat and prevent infections)

  • Continuous follow-up care (to manage disease, detect recurrence of the tumor and to manage late effects of treatment)

Long-term outlook for a child with a brain tumor

Prognosis greatly depends on:

  • The type of tumor

  • The extent of the disease

  • Size and location of the tumor

  • Presence or absence of metastasis

  • The tumor's response to therapy

  • The age and overall health of your child

  • Your child's tolerance of specific medications, procedures, or therapies

  • New developments in treatment

As with any cancer, prognosis and long-term survival can vary greatly from individual to individual. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with a brain tumor. Side effects of radiation and chemotherapy, as well as second malignancies, can occur in survivors of brain tumors.

Rehabilitation for lost motor skill and muscle strength may be required for an extended amount of time. Speech therapists and physical and occupational therapists may be involved in some form of rehabilitation. More research is needed to improve treatment, decrease side effects of the treatment for this disease, and develop a cure. New methods are continually being discovered to improve treatment and to decrease side effects.

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Chemotherapy is the use of anticancer drugs to treat cancer cells.

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John Myseros

John Myseros' Story

Dr. Myseros began at Children’s National more than five years ago and has a personal and professional interest in the care of children with tumors of the brain and spinal cord.

Patient story

Elias's Story Part Three

"As a parent of young children, you know that kids will do the darndest things. So do the best you can, keep your eyes open, but don't beat yourself up if you have to get a new broviac."

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Elias's Story Part Two

"One positive thing I have to say about this process is that the rotation of Children's attending physicians for the oncology inpatient unit is outstanding, and the fellows also are very good. They were accessible (by phone, too, when we were at home), understanding, and of course knowledgeable."

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"I think his medical experiences as a small child helped develop his attitude and approaches for coping. He still thinks of Children's as a friendly, good place, which is a real tribute to the excellent care and treatment he received."

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Mason's Story

"You are about to embark on a journey that will be the challenge of your life as a parent. But you have chosen a hospital and a brain tumor treatment team that is one of the best in the country."

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Amaya's Story

"Amaya is doing great. She has had two MRIs since (every three months) and all is well. She will begin track season healthy and strong!"

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Amaya's Story

Patient story

"Amaya is doing great. She has had two MRIs since (every three months) and all is well. She will begin track season healthy and strong!"

Read More of Amaya's Story