• Germ Cell Brain Tumors in Children

    Germ cell brain tumors develop from germ cells – the cells that later become sperm in the testicles or eggs in the ovaries; during the fetal period, these cells may get “trapped” in the brain. Most germ cell tumors are found in the ovaries (ovarian tumors) or testes (testicular tumors), but when they are located in the brain they are called intracranial germ cell tumors, or germ cell tumors of the brain. Germ cell tumors that develop in the brain or spinal cord also may be called CNS (central nervous system) germ cell tumors.

    • There are two main types of germ cell tumors of the brain – germinomas and non-germinomatous tumors; if they include aspects of both, they are called mixed germ cell tumors.
    • Germ cell tumors of the brain are rare, accounting for approximately 4% of pediatric brain tumors.
    • Around half occur in children between the ages of 10 and 15.
    • Germ cell tumors of the brain most commonly involve or are found near the pituitary and pineal glands in the brain.

    Germ Cell Brain Tumor Treatment at Dana-Farber/Boston Children's

    Children with germ cell brain tumors are treated at Dana-Farber/Boston Children's through the Brain Tumor Center, one of the largest and most experienced pediatric brain tumor treatment programs in the world. Our brain tumor specialists have extensive expertise in treating all types of brain tumors, including germ cell brain tumors. Our patients receive care from neuro-oncologists, neurosurgeons, neurologists, and pediatric subspecialists.

    What are the types of germ cell brain tumors?

    Successfully treating your child’s germ cell brain tumor depends on where the tumor is located and what type of germ cell tumor it is. Doctors at Dana-Farber/Boston Children's will classify your child's tumor as one of the following types:

    • Germinomas, or pure germ cell tumors, are tumors that respond well to treatment.
    • Non-germinomatous germ cell tumors secrete chemicals into the spinal fluid and bloodstream and require more intensive treatment than pure germ cell tumors.
      • There are several sub-types of non-germinomatous germ cell tumor, including teratomas, choriocarcinomas, endodermal sinus tumors (yolk sac tumors), embryonal carcinomas and mixed tumors.

    What are the symptoms and causes of germ cell brain tumors?

    Brain tumors can cause a variety of symptoms in children based primarily on the location of the tumor. Keep in mind that the symptoms of a brain tumor may resemble other more common conditions or medical problems. It is important to consult your child's physician for persistent symptoms.

    Symptoms of germ cell brain tumors typically depend on where they have developed in the brain. For tumors in the pineal gland region, children can have the following symptoms:

    • Hydrocephalus (swelling of the brain)
    • Headache
    • Vomiting
    • Fatigue
    • Behavioral or cognitive changes
    • Uncoordinated body movement (ataxia)
    • Vision changes, including double vision and difficulty looking up

    For tumors in the suprasellar or pituitary gland region, common symptoms include:

    • Diabetes insipidus (an uncommon disorder characterized by intense thirst and the passing of large amounts of urine)
    • Delayed puberty
    • Early (precocious) puberty
    • Stunted growth
    • Vision changes including loss of peripheral vision or decrease in vision

    As a parent, you undoubtedly want to know what may have caused your child’s tumor. 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.

    It’s important to understand that these and other brain tumors most often occur with no known cause. There’s nothing that you could have done or avoided doing that would have prevented the tumor from developing.

    How are germ cell brain tumors diagnosed?

    The first step in treating your child is forming an accurate and complete diagnosis. Germ cell brain tumors are typically diagnosed using a combination of imaging and biopsy.

    As with other brain tumors, diagnostic procedures for germ cell tumors of the brain can determine the exact type of tumor and whether it has spread. Your child’s physician may order a number of different tests including:

    • A physical exam and complete medical history. A neurological function test of reflexes, muscle strength, eye and mouth movement, coordination and alertness.
    • Magnetic resonance imaging (MRI), a diagnostic procedure that produces detailed images of the structures within the brain and spine. An MRI uses a combination of large magnets, radiofrequencies and a computer to analyze organs and structures within the body. No x-rays or radiation are used.
    • Computerized tomography scan (also called a CT or CAT scan), a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images, often called slices, of the body from horizontal and vertical perspectives. CT scans are more detailed than general x-rays.
    • 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 how well the tumor is responding to treatment.
    • Lumbar puncture (spinal tap), to remove a small sample of cerebrospinal fluid (CSF), which can be used to determine if any tumor cells have spread and to analyze tumor markers. In children, this procedure can safely be performed under sedation if needed.

    There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. After we complete all necessary tests, our experts will meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best possible treatment options.

    What are the treatments for germ cell brain tumors?

    Your child's physician will determine a specific course of treatment based on several factors, including your child's age, overall health and medical history, the type, location, and size of the tumor and the extent of the disease.

    Treatments for germ cell brain tumors include:

    • Neurosurgery: Surgery is used preliminarily to obtain a biopsy of the tumor. Tumor specimens are then examined by neuropathologists. After complete diagnosis, depending on the type of germ cell brain tumor, surgeons may do further surgery to remove the tumor completely or in part.
    • Endoscopic third ventriculostomy (ETV) or ventriculo-peritoneal shunt (VP shunt): When a tumor causes blockage of cerebral spinal fluid (CSF) flow, our surgeons may perform one of two procedures in order to relieve symptoms of hydrocephalus, the buildup of fluid inside the skull. In an endoscopic third ventriculostomy, neurosurgeons create a small hole that allows fluid to flow around the blockage. In some cases, children may have an alternative procedure in which a tube is placed in the ventricles to the abdomen to drain excess fluid into the abdomen. This is called a VP shunt.
    • Radiation therapy: High-energy waves from a specialized machine damage or kill cancer cells and shrink tumors. Tumors that are not likely to spread receive radiation only to the tumor and the area surrounding it. If the tumor is likely to spread beyond its original location, radiation to other parts of the brain and spinal cord may be recommended. If the tumor has already spread, radiation is given to the whole brain and spinal cord.
    • 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. Your child may receive chemotherapy orally, as a pill to swallow; intramuscularly, as an injection into the muscle or fat tissue; intravenously, as a direct injection into the bloodstream, or IV; or intrathecally, as a direct injection into the spinal column through a needle

    What is the latest research on germ cell brain tumors?

    Clinical trials, or research studies evaluating new treatment approaches, are a major offering at Dana-Farber/Boston Children’s. Clinical trials are very important for children with hard-to-treat or relapsed conditions.

    In addition to launching our own brain tumor clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG) and the Pacific Neuro-oncology Consortium (PNOC). We are also the New England Phase I Center of the Children's Oncology Group. If your child has progressive or recurrent tumor, she may be eligible for a number of experimental therapies available through these groups, or from one of our independent clinical investigators.

    Clinical Trials

    For many children with brain tumors or other rare or hard-to-treat conditions, clinical trials provide new options.

    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.

    Contact us: If you’re not sure which clinical trials might be right for your child, email us at clinicaltrials@danafarberbostonchildrens.org. We can help you navigate your options.

    What is the long-term outlook for children with germ cell brain tumors?

    The prognosis for children with germ cells tumor of the brain largely depends on the type of tumor.

    In general, germinomas are cured in greater than 90 percent of cases with combined treatment. Non-germinomatous germ cell tumors have a 65 percent to 85 percent cure rate, depending on their spread at diagnosis. Mixed germ cell brain tumors are generally more difficult to treat.

    Children treated for a germ cell tumor in the brain should visit a survivorship clinic every year to manage disease complications, screen for recurrence and manage late treatment side effects. A typical follow-up visit is likely to include a physical exam, laboratory testing and imaging scans.

    At our brain tumor survivorship program – the Stop and Shop Family Pediatric Neuro-Oncology Outcomes Clinic – children meet with their neurosurgeon, radiation oncologist, pediatric neuro-oncologist and neurologists at the same follow-up visit. Our multidisciplinary approach and depth of expertise also give your child on-site access to endocrinologists, neuro-psychologists and alternative/complementary therapy specialists. 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.

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