Dysembryoplastic neuroepithelial tumor (DNT)

  • If your child has been experiencing seizures that don’t respond to medication, a possible cause may be a dysembryoplastic neuroepithelial tumor (DNT). This is a rare, benign type of tumor that occurs in the tissues that cover the brain and spinal cord. The outlook for a child with DNT is generally good.

    As you read further below, you will find general information about dysembryoplastic neuroepithelial tumors (DNT). To read summary information about brain tumors first, see the overview on brain tumors.

    How Dana-Farber/Boston Children’s Cancer and Blood Disorders Center approaches DNT:

    If your child is cared for at Children’s, he’ll be seen through Dana-Farber/Children’s Hospital Cancer Center, an integrated pediatric oncology program through Dana-Farber Cancer Institute and Children’s Hospital Boston that provides—in one specialized program—all the services of both a leading cancer center and a pediatric hospital.

    Our pediatric neuro-oncology, neurosurgical and neurology specialists at Dana-Farber/Children’s Hospital Cancer Center offer:

    • technological advances, such as the intra-operative MRI, which allow our pediatric neurosurgeons to “see” the tumor as they operate with MRI scans. This allows them to remove as much of the tumor as possible.
    • treatment with the best standard of care, including neurosurgery, radiation therapy and chemotherapy
    • access to unique Phase I clinical trials run by our own investigators, Children’s Oncology Group and the Pediatric Oncology Experimental Therapeutics Consortium

    Through the Stop and Shop Neuro-Oncology Outcomes Clinic at Dana-Farber Cancer Institute, your child will be are able to meet with his entire care team at the same follow-up visit.

    • Our pediatric brain tumor survivorship clinic is held weekly.
    • In addition to meeting with your pediatric neuro-oncologists, neurologist and neurosurgeon, your child may also see one of our endocrinologists or alternative/complementary therapy specialists.
    • School liaisons and psychosocial personnel from the pediatric brain tumor team are also available.
    • If your child needs rehabilitation, he may also meet with speech, physical, and occupational therapists during and after treatments.
  • In-Depth

    Who is at risk for a DNT?

    A DNT usually begins in children and teenagers who are 20 years old or younger.

    What are the symptoms of dysembryoplastic neuroepithelial tumor (DNT)?

    While each child may experience symptoms differently, and symptoms may vary depending on the size and exact location of the tumor, the most common symptom for DNT is the presence of seizures that are difficult to control with anti-seizure medication. In fact, it’s not uncommon for children to have their first seizure before age 10 and then continue to experience them for several years before the diagnosis is made.

    DNT tumors are found in the cerebrum, which is the part of the brain that controls thought, movement and sensation, so your child may experience other symptoms that relate to those functions.

    What is a DNT made of? 

    These tumors consist of different types of abnormal cells including:

    • oligodendrocytes (cells that provide support and nourishment for cells that transmit nerve impulses)
    • neurons
    • astrocytes (connective tissue cells)
  • If your child’s doctor suspects that he may have a dysembryoplastic neuroepithelial tumor, she may order diagnostic tests including:

    • neurological exam– a test of your child’s reflexes, muscle strength, eye and mouth movement, coordination and alertness
    • computerized tomography scan (also called a CT or CAT scan)– This is a diagnostic procedure that uses x-rays and computer technology to produce cross-sectional images (often called slices), of the brain and spinal cord. A CT scan will identify low-density dysembryoplastic neuroepithelial tumors.
    • magnetic resonance imaging (MRI)– This is a procedure that uses large magnets, radiofrequencies and a computer to produce detailed images of your child’s organs and other bodily structures. It can help confirm the presence of a DNT and distinguish it from other types of tumors.
    • immunohistochemical and ultrastructural studies- lab-based studies that can confirm the origin of DNT and help your child’s doctor determine the best treatment
  • At Boston Children's Hospital, we know how difficult a diagnosis of a brain tumor 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.

    The most common treatment for DNT is to remove the tumor surgically. Because it is a benign tumor, and prognosis is good even if not the entire tumor is not removed, radiation and chemotherapy are not used. 

    What's the long-term outlook for my child? 

    The outlook for dysembryoplastic neuroepithelial tumors (DNT) is uniformly good, regardless of how much of the tumor is removed.

  • A hopeful future

    Treatment for brain tumors in children has progressed tremendously in the last decade:

    • New tools are being used to help doctors diagnose tumors sooner and with more accuracy.
    • Radiation therapy and chemotherapy are increasingly targeting tumors more accurately and effectively while keeping clear of healthy brain cells and tissue.
    • A successful new surgical technique is the intra-operative MRI, which gives surgeons a three-dimensional picture of the tumor so they can remove the cancer while leaving other parts of the brain relatively untouched.

    Research & clinical trials

    Our program offers unique access to a range of clinical trials in which your child can receive the newest brain tumor treatments. Through this research, our physicians work to improve current therapeutic approaches and outcomes for many hard-to-treat pediatric brain tumors.

    Dana-Farber/Boston Children’s Cancer and Blood Disorders Center oversees New England’s most active pediatric oncology clinical research program; it provides access to unique clinical trials for patients with newly diagnosed, relapsed or refractory brain tumors. We are:

    • New England’s Phase I Children’s Oncology Group referral center, a Pediatric Oncology Experimental Therapeutics Investigator Consortium member (POETIC), and a founding institution of the Pediatric Brain Tumor Consortium. We are also members of the Pediatric Blood and Marrow Transplant Consortium (PBMTC)
    • skilled at improving current therapeutic approaches and outcomes of hard to treat pediatric brain tumors.

    Rapidly translating scientific discoveries to the bedside is a major focus of the program. Members of our brain tumor team:

    • played a key role in the identification and application of anti-angiogenic treatments for pediatric brain tumors
    • launched new studies investigating gene profiling of patients’ tumors and the development of personalized treatment approaches
    • pioneered the development of new treatments for specific highly malignant tumors such as ATRT
    • are developing tissue registries to better classify and treat certain types of brain tumors
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