Germ Cell Tumors of the Brain

  • What is a germ cell tumor of the brain?

    Germ cell tumors of the brain develop when the sex cells that should normally form the ovaries or testes fail to migrate down and instead become “trapped” in the brain.  This is why most germ cell tumors are found in the testes or ovaries. When found in the brain, they’re known as “intracranial germ cell tumors”.

     Here are some basics about germ cell tumors of the brain:

    • Germ cell tumors in the brain are most commonly found in the areas around the pituitary and pineal glands.
    • Germ cell tumors in the brain are very rare, accounting for approximately 4 percent of all pediatric brain tumors.
    • Around half of these tumors occur in young people between ages 10 and 20.
    • Most germ cell tumors are classified as malignant (cancerous).
    • Treatment for germ cell tumors can include surgery, radiation and chemotherapy.

    As you read further, you will find general information about germ cell tumors in the brain. If you would like to view summary information about brain tumors first, see the overview on brain tumors.

    How Dana-Farber/Boston Children’s Cancer and Blood Disorders Center approaches germ cell tumors of the brain

    We understand that you may have a lot of questions when your child is diagnosed with a germ cell tumor of the brain. Is it dangerous? Will it affect my child long-term? What do we do next? We’ve tried to provide some answers to those questions in the following pages, and our experts can explain your child’s condition fully.

    We hold a weekly brain tumor clinic for newly diagnosed patients currently receiving treatment. Each time you come for an appointment, you meet with specialists on your child’s team, from your pediatric neuro-oncologist, neurologist and neurosurgeon, to your pediatric endocrinologist, and psycho-oncologis.


     

  • A diagnosis of a germ cell tumor of the brain comes with a lot of questions and uncertainty about your child’s health, like:

    • Exactly what type of tumor does my child have?
    • How is my child going to be treated?
    • What’s the long-term outlook for my child?

    At Boston Children’s Hospital, we know that these concerns are entirely natural.  To help put your mind as ease, we’ve provided answers to many commonly asked questions about germ cell tumors of the brain in the following pages. When you meet with our team of doctors, they’ll be able to explain your child’s condition and treatment options fully.

    What are germ cell tumors?

    • Germ cell tumors are tumors that begin in germ cells, or cells that can develop into the reproductive cells in the body. When two germ cells combine, they form the fetus, the placenta and then all the organs.
    • Germ cell tumors are a widely varied group of tumors. They range from very low grade or benign to highly malignant or aggressively growing cancers.

    What are the different types of germ cell tumors in the brain?

    Pure germ cell tumors

    • Pure cell tumors are very immature (underdeveloped) tumors. They rarely secrete any chemicals into the bloodstream.
    • These tumors respond well to therapy.
    • Pure cell tumors have a 90 percent cure rate.

    Mixed germ cell tumors

    • Mixed germ cell tumors (also known as nongerminomatous germ cell tumors) require more aggressive therapy than pure germ cell tumors.
    • Mixed germ cells secrete chemicals into the blood stream that can be detected inblood and spinal fluid tests.
    • Mixed germ cell tumors contain cancerous, or malignant, forms of these tumors, including:
    • embryonal carcinoma
    • choriocarcinoma
    • endodermal sinus [yolk sac] tumors
    • Mixed germ cell tumors have a 70 – 75 percent cure rate

    Teratoma

    • In mixed germ cell tumors, there can be a teratoma component and a malignant component.
    • Benign teratomas can grow aggressively, although they do not spread.
    • In Malignant teratomas (such as one with an embryonal carcinoma), the malignant componentcan grow aggressively and spread to other parts of the body.
    • In malignant teratomas chemotherapy and radiation treat the malignant component, but the teratoma component sometimes does notrespond well to chemotherapy and additional surgery is sometimes necessary to remove the remaining tumor.  

    Causes

    What causes a germ cell tumor of the brain?

    The cause of germ cells tumors is not well known. Usually, germ cells migrate to the gonads during fetal development and become an egg in the female ovaries or sperm in the male testes. However, when these germ cells don’t move to the right area, they become trapped in the brain and multiply in areas where they shouldn’t.

    Signs and symptoms

    Symptoms vary depending on size and location of tumor. 

    For germ cell tumors located in the pineal gland region, symptoms may include:

    • hydrocephalus
    • headache
    • vomiting
    • fatigue
    • behavioral or cognitive changes
    • uncoordinated body movements (ataxia)
    • visual changes, including double vision and difficulty looking up

    For germ cell tumors found in the suprasellar region or pituitary gland, symptoms might include:

    The symptoms of a brain tumor may resemble other conditions or medical problems, ranging from the simple to the serious. Always consult your child's physician for diagnosis and treatment.

    Questions to ask your doctor

    If your child has just been diagnosed with a germ cell tumor of the brain, you probably have a lot of question and concerns on your mind. When you meet with your child’s doctor, it can be easy to be overwhelmed with information and forget the questions you wanted to ask.

    A lot of parents find it helpful to jot down questions beforehand. That way, when you talk to your child’s clinician, you can be sure that all your concerns are addressed. Remember that physicians are open to learning from families too. Attend conferences, read up on updated materials and don’t be afraid to share what you have learned.

    Some questions you might ask include:

    • How will my child’s tumor be treated?
    • What are the long-term effects of these treatments?
    • How should I explain the brain tumor to my child’s siblings?
    • Where can I go for emotional support?
    • What other resources can you point me to for more information?
  • The first step in treating your child is forming an accurate and complete diagnosis. This typically begins with:

    • a complete medical history
    • a physical examination

    How are germ cell tumors in the brain diagnosed?

    Diagnostic procedures for germ cell tumors, like other brain tumors, determine the exact type of tumor and whether the tumor has spread.

    These may include:

    • A  physical exam to test neurological function including: reflexes, muscle strength, eye and mouth movement, coordination, and alertness.
    • Magnetic resonance imaging (MRI) This is an imaging procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body.  There are no radiation or x-rays used in MRI scans
    • Computerized tomography scan (also called a CT or CAT scan) is a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. CT scans are more detailed than general x-rays.

    After your child’s doctors suspect a tumor, they’ll begin the process of figuring out what kind of tumor it is. These may include:

    • Blood tests to check for “tumor markers”. Certain germ cell tumors release measurable substances into the blood such as alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (B-HCG). These tumor markers can be repeatedly tested in order to track the how well the tumor is responding to treatment.
    • Lumbar puncture (spinal tap) to remove a small sample of cerebrospinal fluid (CSF) and determine if any tumor cells have started to spread and analysis for tumor markers. In young children, this procedure can safely be performed under sedation.

    In order to determine the best treatment for your child’s tumor, the doctors may need even more information about it. In that case, they may do a biopsy to get a tiny sample of tumor tissue to study under the microscope.

    • A biopsy is a tissue sample from the tumor that provides definitive information about the type of tumor. This is collected during surgery.
  • At Boston Children's, we know how difficult a diagnosis of germ cell tumors can be, both for your child and for your whole family. That's why our physicians are focused on family-centered care: From your first visit, you'll work with a team of professionals who are committed to supporting all of your family's physical and psychosocial needs. We'll work with you to create a care plan that's best for your child. 

    What are the treatments for a germ cell tumors of the brain?

    Treatment may include the following, alone or in combination.

    Surgery

    Usually first step of treatment is surgery: a pediatric neurosurgeon removes as much of the tumor as possible while preserving your child's neurological function. Tumor specimens are examined by neuropathologists.

    • Surgery is usually limited to well encapsulated teratomas, a particular tumor that includes all three cellular layers of germ cells, or in the case of blocked CSF flow.
    • Complete resection or surgical removal of the entire tumor is ideal when possible. However, tumor location and other characteristics may limit removal to a partial or sub-total resection.
    • A biopsy is the surgical removal of a sample of the tumor for diagnostic purposes. This is frequently done if the tumor is in an area with sensitive structures around it that may be injured during removal.

    Ventriculo-peritoneal shunt (VP shunt)

    When a tumor causes blockage of cerebral spinal fluid (CSF) flow, special tubing can be surgically implanted in the ventricles to drain excess CSF into the abdomen. This bypasses the tumor blockage and relieves symptoms of hydrocephalus, the build up of fluid inside the skull.

    Radiation therapy

    Precisely targeted and dosed radiation is used to kill cancer cells left behind after surgery. This treatment is important to control the local growth of tumor.

    • Tumors that are not likely to spread receive radiation only to the tumor and the area surrounding it.
    • If we determine the tumor is more likely to spread beyond its original tumor location, radiation to other parts of the brain and spinal cord may be recommended.
    • If the tumor has spread, radiation to the whole brain and spinal cord are used to treat certain germ cell tumors.

    Chemotherapy

    Chemotherapy is a drug that interferes with the cancer cell's ability to grow or reproduce. Chemotherapy before surgery may help shrink the tumor, making it possible to remove.

    • Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.
    • Often, a combination of chemotherapy drugs is used.
    • Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat.

    While chemotherapy can be quite effective in treating certain cancers, the agents don't differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.

    Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells.

    Chemotherapy can be given:

    • as a pill to swallow.
    • as an injection into the muscle or fat tissue.
    • Intravenously, directly to the bloodstream (also called IV).
    • Intrathecally, which means directly delivering the chemotherapy into the spinal column with a needle.

    What is the expected prognosis after treatment for my child?

    Outcomes vary widely, depending on the precise type of germ cell tumor your child has. In general, germinomas are cured in greater than 85 percent of cases with combined treatment.

    • Mature teratomas are curable with complete resection alone.
    • Immature teratomas usually require additional therapy.
    • Other germ cell tumors, the mixed and malignant types, are more difficult to treat.

    What is the recommended long-term care for my child? 

    Children treated for a germ cell tumor in the brain should visit a survivorship clinic every year to:

    • manage disease complications
    • screen for early recurrence of tumor
    • manage late effects of treatment

    A typical follow-up visit may include some or all of the following:

    • a physical exam
    • laboratory testing
    • imaging scans

    Through the Stop and Shop Family Pediatric Neuro-Oncology Outcomes Clinic as Dana-Farber Cancer Institute, children are able to meet with their neurosurgeon, radiation oncologist, pediatric neuro-oncologist and neurologists at the same follow-up visit.

    • Endocrinologists, neuro-psychologists, alternative/complementary therapy specialists, and school liaison and psychosocial personnel from the pediatric brain tumor team are also available.
    • In addition, children needing rehabilitation may meet with speech, physical, and occupational therapists during and after treatments.
  • What is the latest research on germ cell tumors?

    Dana-Farber/Boston Children’s Cancer and Blood Disorders Center is a member of the Pediatric Oncology Therapeutic Experimental Investigators Consortium (POETIC), a collaborative clinical research group offering experimental therapies to patients with relapsed or refractory disease.

    Participation in any clinical trial is completely voluntary: We will take care to fully explain all elements of the treatment plan prior to the start of the trial, and you may remove your child from the medical study at any time.

    Find a clinical trial

    For more information on current research, see the Brain Tumor Program.

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