Desmoplastic infantile ganglioglioma (DIG) is a rare type of childhood brain tumor. A tumor occurs when cells in the body become abnormal and multiply without control or order. DIG is a type of glioma, a tumor that arises from the glial cells, which are supportive cells in the brain. It usually occurs on one side of the brain, can be very large, and is usually found in children younger than one year old. DIG is comprised of more than one type of cell and also contains cysts. It is thought that DIG begins before a baby is even born.
DIG was originally classified as a low-grade, slow-growing tumor that is usually benign (noncancerous). However, in some cases, the tumor grows more rapidly and is more aggressive in spreading to other parts of the brain or body.
A risk factor is anything that increases a person’s chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop the disease, while others with no known risk factors do.
The causes of DIG are unknown. There are no known risk factors or ways to prevent the disease.
Children with DIG may experience the following symptoms. Sometimes, children with DIG do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a tumor. If you are concerned about a symptom on this list, please talk with your child’s doctor.
Doctors use many tests to diagnose a tumor and determine if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For tumors, a biopsy is the only way to make a definitive diagnosis. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the tumor has metastasized. Your doctor may consider these factors when choosing a diagnostic test:
Age and medical condition
The type of cancer suspected
Severity of symptoms
Previous test results
In addition to a physical examination, the following tests may be used to diagnose DIG:
Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a vein to provide better detail.
Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture.
Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).
Staging is a way of describing a tumor, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the tumor's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of tumors.
There is no standard staging system for DIG; however, it is classified by the World Health Organization (WHO) under a grade I nervous system tumor.
WHO grade I: Includes distinct, unconnected tumors that are less likely to spread and can often be successfully treated with only the surgical removal of the tumor.
WHO grade II: Includes tumors that are likely to spread and have low numbers of dividing tumor cells that may come back after treatment. Some tumor types tend to progress to a higher grade.
WHO grade III: Includes tumors that have malignant (cancerous) characteristics, such as dividing tumor cells, evidence of spreading, and undifferentiated cells (look less like normal cells when viewed under a microscope).
WHO grade IV: Includes tumors that have actively dividing tumor cells, areas of tumor cell death, and generally grow rapidly both before and after surgery.
Recurrent: A recurrent tumor is a tumor that comes back after treatment.
Clinical trials are the standard of care for the treatment of children with cancer. In fact, more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments (the best treatments available) to newer treatments that may be more effective. Cancer in children is rare, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. Investigating new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track progress.
To take advantage of these newer treatments, all children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. Many times, a team of doctors treats a child with cancer. Pediatric cancer centers often have extra support services for children and their families, such as nutritionists, social workers, and counselors. Special activities for kids with cancer may also be available.
The treatment of DIG depends on the size and location of the tumor, whether the tumor has spread, and the child’s overall health.
Surgery
Surgery is the main treatment for DIG, with the goal being the complete removal of the tumor. Sometimes, surgery cannot be performed because the tumor cannot be reached or is near a vital structure. After surgery, the treatment plan is determined based on the following criteria:
The amount of tumor removed
The age of the child
The presence of metastases
Chemotherapy
Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy may be used in some cases to slow or stop the growth of DIG when surgery is not an option. It also may be given before surgery to shrink the tumor or to destroy any tumor remaining after surgery. The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.
The medications used to treat cancer are continually being evaluated. Talking with your child’s doctor is often the best way to learn about the medications prescribed for your child, their purpose, and their potential side effects or interactions with other medications. Learn more about your child’s prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.
Doctors and scientists are always looking for better ways to treat patients with DIG. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.
People decide to participate in clinical trials for many reasons. For some people, a clinical trial is the best treatment option available. Because standard treatments are not perfect, people are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other people volunteer for clinical trials because they know that finding new drugs and other therapies is the only way to make progress in treating DIG. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with DIG.
In order to join a clinical trial, people must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different than the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.
A tumor and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.
Fear of treatment side effects is common after a diagnosis of DIG, but it may be helpful to know that preventing and controlling side effects is a major focus of your child’s health-care team. Before treatment begins, talk with your child’s doctor about possible side effects of the specific treatments your child will be receiving. The specific side effects that can occur depend on a variety of factors, including the size and location of the tumor, the individual treatment plan (including the length and dosage of treatment), and your child’s overall health.
Ask your child’s doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health-care team if they do happen. Also, be sure to communicate with the doctor about side effects your child experiences during and after treatment. For more information on the most common side effects of a tumor and different treatments, along with ways to prevent or control them, visit Cancer.Net’s section on Managing Side Effects, based on ASCO’s curriculum.
In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing these needs in Cancer.Net’s section on Caring for the Whole Patient.
For more information on late effects or long-term side effects, please read the After Treatment section or talk with your child’s doctor.
After treatment for DIG ends, talk with your child’s doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your child’s recovery for the coming months and years. All children treated for cancer, including DIG, should have life-long, follow-up care.
Specific after-treatment care must be determined on a case-by-case basis, because there are many factors that can affect the outcome of treatment for DIG, such as tumor location, whether the tumor could be removed, the need for and type of postoperative (after surgery) therapy, and age at the time of treatment. Postoperative follow-up care with MRI scans is routinely performed to monitor for signs of tumor recurrence or growth.
Based on the type of treatment the child received, the doctor will determine what examinations and tests are needed to check for long-term side effects and the possibility of secondary cancers. Your child’s doctor can recommend the necessary screening tests. Follow-up care should also address the child’s quality of life, including any developmental or emotional concerns. Learn more about Childhood Cancer Survivorship.
The child’s family is encouraged to organize and keep a record of the child’s medical information, so that as the child enters adulthood, he or she has a clear, written history of the diagnosis and details of the treatment given. The doctor’s office can help you compile this, and it should include recommendations from the doctor about the schedule for follow-up care during his or her lifetime.
Children who have had cancer can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with your doctor about developing a plan that is best for your child’s needs.
Research for DIG is ongoing. Various advances may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your child’s doctor.
Regular communication with your child’s doctor is important for making informed decisions about his or her health care. Consider asking the following questions of your child’s doctor:
What is the grade of the tumor? What does this mean?
Can you explain my child’s pathology report to me?
What is your familiarity with my child's tumor type and its treatment?
What treatment options are available?
What clinical trials are open to my child?
What treatment do you recommend? Why?
What is the chance for success with each treatment option?
What are the possible side effects of this treatment, both in the short term and the long term?
How will this treatment affect my child’s daily life?
What follow-up tests will my child need, and how often will he or she need them?
What support services are available to my child? To my family?
Alliance for Childhood Cancer 1900 Duke St., Ste. 200
Alexandria, VA 22314
Phone: 703-299-1050 www.childhoodcanceralliance.org
American Brain Tumor Association
2720 River Rd.
Des Plaines, IL 60018
Phone: 847-827-9910
Toll Free: 800-886-2282 www.abta.org
Beyond the Cure
1 South Memorial Dr., Ste. 800
St. Louis, MO 63102
Toll Free: 800-5-FAMILY (532-6459) www.beyondthecure.org
The Brain Tumor Foundation
1350 Avenue of the Americas, Ste. 1200
New York, NY 10019
Phone: 212-265-2401 www.braintumorfoundation.org
The Brain Tumor Society
124 Watertown Street, Ste. 3-H
Watertown, MA 02472
Phone: 617-924-9997
Toll Free: 800-770-TBTS (8287) www.tbts.org
Candlelighters Childhood Cancer Foundation
P.O. Box 498
Kensington, MD 20895
Phone: 301-962-3520
Toll Free: 800-366-2223 www.candlelighters.org
The Childhood Brain Tumor Foundation
20312 Watkins Meadow Dr.
Germantown, MD 20876
Phone: 301-515-2900
Toll Free: 877-217-4166 www.childhoodbraintumor.org
Children's Brain Tumor Foundation 274 Madison Ave., Ste. 1004
New York, NY 10016
Toll Free: 866-228-4673 www.cbtf.org
The Children's Cause for Cancer Advocacy
1010 Wayne Ave., Ste. 770
Silver Spring, MD 20910
Phone: 301-562-2765 www.childrenscause.org
Children's Hospice International
1101 King St., Ste. 360
Alexandria, VA 22314
Toll Free: 800-24-CHILD (800-242-4453)
Phone: 703-684-0330 www.chionline.org
CureSearch
4600 East West Hwy., Ste. 600
Bethesda, MD 20814
Toll Free: 800-458-6223 www.curesearch.org
Make-A-Wish Foundation
3550 N Central Ave., Ste. 300
Phoenix, AZ 85012
Phone: 602-279-WISH (9474)
Toll Free: 800-722-WISH (9474) www.wish.org
National Brain Tumor Foundation
22 Battery St., Ste. 612
San Francisco, CA 94111
Phone: 415-834-9970
Toll Free: 800-934-CURE (2873) www.braintumor.org
National Children's Cancer Society
1 South Memorial Dr., Ste. 800
St. Louis, MO 63102
Phone: 314-241-1600
Toll Free: 800-532-6459 www.children-cancer.com
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