Dysembryoplastic Neuroepithelial Tumor (DNET) or Ganglioneuroma
What is a Dysembryoplastic Neuroepithelial Tumor (DNET) or Ganglioglioma?
DNET or ganglioglioma are rare, slow growing tumors that account for less than two percent of brain tumors. They typically develop in the temporal lobe or frontal lobe of the brain, and are rarely located in other areas of the brain and spinal cord. These tumors do not have a capacity to metastasize or spread beyond the primary site of origin. DNET or ganglioglioma are mainly seen in children and young adults and the average age of diagnosis is 9 years of age. There is no known cause for DNET and ganglioglioma.
What are the symptoms of Dysembryoplastic Neuroepithelial Tumor (DNET) or Ganglioglioma?
Usually the episode of a seizure is the first indication something is wrong and can be accompanied by headache, vomiting, difficulty with balance, and lethargy. Ganglioglioma of the brainstem and spinal cord may also causeweakness, scoliosis, back pain or bowel and bladder dysfunction.
How is a Dysembryoplastic Neuroepithelial Tumor (DNET) or Ganglioglioma diagnosed?
Several tests are used to diagnose a DNET or ganglioglioma. Patients who experience seizures undergo an electroencephalogram (EEG) to locate the origin of the seizure. If a brain tumor is detected, a magnetic resonance imaging (MRI) of the brain and/or spine can further define the location of the tumor. A functional MRI or magnetoencephalogram (MEG) maps out the important brain function located around the tumor, which will ultimately help the surgeon with precise removal of the tumor. A biopsy of the tumor is required to make the final diagnosis of a DNET or ganglioglioma.
How is a Dysembryoplastic Neuroepithelial Tumor (DNET) or Ganglioglioma treated?
Surgery is the treatment for DNET and ganglioglioma, with the goal to completely remove the tumor. Complete tumor removal is associated with a very high cure rate. Surgical removal of the tumor also helps control seizures. The majority of patients treated for a DNET and ganglioglioma are cured of their disease following the first surgery. Chemotherapy or radiation therapies are not necessary in a typical DNET or ganglioglioma.
About treatment for Dysembryoplastic Neuroepithelial Tumor (DNET) or Ganglioglioma at Children’s Hospitals and Clinics of Minnesota
Our cancer and blood disorders program consistently achieves excellent results ranking it in the top ten programs in the United States. Children’s Hospitals and Clinics of Minnesota treats the majority of children with cancer and blood disorders in Minnesota and provides patients access to a variety of clinical trials using ground-breaking new treatments. Through our renowned program, patients experience unparalleled family support, a nationally recognized pain management team, and compassionate, coordinated care.
If you are a family member looking for a Children’s neuro-oncologist, please call our clinic at (612) 813-5940.
If you are a health professional looking for consultation or referral information, please call Children's Physician Access at 1-866-755-2121 (toll-free) and ask for the on-call hematologist/oncologist.
Additional information on Dysembryoplastic Neuroepithelial Tumor (DNET) or Ganglioglioma
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