What Is a Childhood Dysembryplastic Neuroepithelial Tumor?
A dysembryplastic neuroepithelial tumor (DNET, or DNT) is a benign (non-cancerous), slow-growing brain tumor. It is a glioneuronal tumor, which means it contains both glial cells (responsible for providing the structural support of the central nervous system) and neuronal cells (the functioning component of the central nervous system).
- DNET often causes seizures that don't respond to medication.
- DNET occurs in the tissues that cover the brain and spinal cord.
- The tumor usually begins in children and individuals who are 20 years old or younger.
At Dana-Farber/Boston Children's Cancer and Blood Disorders Center, our brain tumor specialists have expertise in treating all types of glial and neural tumors, including DNET. Children receive world-class care from a team of neuro-oncologists, neurosurgeons, and pediatric cancer experts in our Childhood Glioma Program, which is part of our Brain Tumor Center.
Symptoms of Childhood DNET
Seizures that are difficult to control with anti-seizure medication are the most common DNET symptom. In some cases, a child will have their first seizure before age ten and continue experiencing them for a few years until a DNET diagnosis.
DNET tumors occur in the cerebrum, which is the part of the brain that controls thought, movement, and sensation. Some children will experience other symptoms that relate to those functions.
How We Diagnose Childhood DNET
If your child’s doctor suspects DNET, they will order various diagnostic tests to confirm the diagnosis. Tests may include a comprehensive neurological exam, imaging studies, and lab studies to determine the origin of DNET.
At Dana-Farber/Boston Children’s, we bring together an expert team to review the results and develop a recommended treatment plan. Your doctor will meet with you and your family to discuss treatment options and next steps.
How We Treat Childhood DNET
Surgery is the most common DNET treatment. If surgeons can’t remove the entire tumor, a child’s prognosis is still good because DNET is benign. We do not use radiation therapy or chemotherapy to treat DNET.
Once treatment is complete, we continue to care for children and their families through our pediatric cancer survivorship programs, including the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic for pediatric brain tumor survivors.
Long-term Outcomes for Children with DNET
The outlook for children with DNET is uniformly good, regardless of how much of the tumor has been removed.