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My Child Has:
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Dysembryoplastic Neuroepithelial Tumor(DNT)
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The term dysembryoplastic neuroepithelial tumor (DNT) describes a group of tumors that occur in the tissues that cover the brain and spinal cord. These complex tumors are found in patients with long-standing partial complex seizures. These are supratentorial tumors, meaning they occur in the cerebrum, the part of the brain that controls thought, movement and sensation. These tumors can begin in individuals from 1 to 19 years old. Two-thirds of these tumors arise in the temporal lobe (the side temples of the skull), one third in the frontal lobe (the largest part of the cerebral cortex).
As you read further below, you will find general information about dysembryoplastic neuroepithelial tumors (DNT). If you would like to view summary information about brain tumors first, see the overview on brain tumors.
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Each child may experience symptoms differently. Symptoms vary depending on size and location of tumor. The most common symptom for dysembryoplastic neuroepithelial tumors (DNT) is the occurrence of temporal lobe seizures, often difficult to control with anti-seizure medication. For many patients, these seizures will begin in the first decade of life and continue for several years before the diagnosis of DNT is made.
The symptoms of a brain tumor may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
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These tumors consist of abnormal oligodendrocytes (cells that provide support and nourishment for cells that transmit nerve impulses), neurons, and, less prominently, astrocytes (connective tissue cells). Normal processes of cell division are absent. Advanced examination of these cells in a laboratory, called immunohistochemical and ultrastructural studies, confirm the origin of DNT and help physicians determine the best treatment.
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Diagnostic procedures for a dysembryoplastic neuroepithelial tumor (DNT) may include:
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- neurological exam - your child's physician tests reflexes, muscle strength, eye and mouth movement, coordination, and alertness.
- 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), both horizontally and vertically, of the body, including the brain and spinal cord. A CT scan will identify low-density dysembryoplastic neuroepithelial tumors.
- magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. This test can help confirm the presence of a dysembryoplastic neuroepithelial tumor and distinguish it from other types of tumors.
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Specific treatment for dysembryoplastic neuroepithelial tumors (DNT) will be determined by your child's physician based on:
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- 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
- how your child's doctors expects the disease to progress
- your opinion or preference
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Surgical removal of the tumor is the treatment of choice for dysembryoplastic neuroepithelial tumors (DNT). Radiation therapy and chemotherapy are not used for this type of tumor. The majority of patients can undergo surgical removal, however, even when this is not possible, radiation therapy is not called for because patients rarely have tumor progression, regardless of the extent of the surgery.
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The prognosis for dysembryoplastic neuroepithelial tumors (DNT) is uniformly good regardless of the extent of surgical removal of the tumor.
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Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help clinicians better understand and treat dysembryoplastic neuroepithelial tumors (DNT). For more information on current research, see the Brain Tumor Program.
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