Desmoplastic Infantile Ganglioglioma - Childhood Tumor

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What is Desmoplastic Infantile Ganglioglioma - Childhood Tumor ? 

Desmoplastic infantile ganglioglioma (DIG) is a rare type of brain tumor that can occur during childhood. A tumor begins when normal cells change and grow uncontrollably, forming a mass. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). DIG is a type of glioma, a tumor that starts in 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 more common for children younger than one year old. DIG is made up of more than one type of cell and also contains cysts. It is thought that DIG begins even before a baby is born.

DIG was originally classified as a low-grade, slow-growing tumor that is usually noncancerous. However, in some instances, the tumor grows more rapidly and is more aggressive in spreading to other parts of the brain or body; this is called a high-grade tumor.

Symptoms and Signs

Children with DIG may experience the following symptoms or signs. 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 or sign on this list, please talk with your child’s doctor.

Seizure

Rapid skull growth/increased head size

Tense and bulging fontanelle (infant’s soft spot)

Abnormal sensations

Decreased sensation

Numbness

Weakness

Loss of muscle control

Increased or decreased muscle tone

Paralysis

Your child’s doctor will ask you questions about the symptoms your child is experiencing to help find out the cause of the problem, called a diagnosis. This may include how long he or she has been experiencing the symptom(s) and how often.

If a tumor is diagnosed, relieving symptoms and side effects remains an important part of care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your child’s health care team about symptoms your child experiences, including any new symptoms or a change in symptoms.

Risk Factors

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of cancer, most do not directly cause a tumor. 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.

Diagnosis

Doctors use many tests to diagnose a tumor and find out if it is cancerous, and if so, if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For a tumor, 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 spread. Your child’s doctor may consider these factors when choosing a diagnostic test:

Age and medical condition

Type of tumor 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 during the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).

After these diagnostic tests are done, your child’s doctor will review all of the results with you. If the diagnosis is a tumor, these results also help the doctor describe the tumor; this is called staging.

Stages and Grades

Staging is a way of describing where a tumor is located, if or where it has spread, and whether 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) as a grade I nervous system tumor. The four-stage (I to IV) WHO system is described below:

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. (This is the grade given to DIG by the WHO.)

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 (become more aggressive).

WHO grade III: Includes tumors that have cancerous characteristics, such as dividing tumor cells, evidence of spreading, and undifferentiated cells (cells that 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. If there is a recurrence, the tumor may need to be graded again using the system above.

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