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Neurology

Neuronal Brain Tumors

Neuronal brain tumors are a diverse group of primary central nervous system tumors consisting of lesions with either a primarily neuronal background or a mixed glial and neuronal background, termed glioneuronal tumors. These are rare tumors most commonly associated with epilepsy, although other symptoms may also occur, such as headaches, focal neurological deficits, and acute hydrocephalus. This activity reviews the evaluation and treatment of neuronal brain tumors. It highlights the role of the

v1 · Reviewed Nov 7, 2026 by Automated Clinical Reviewer (Strict Verbatim v1)
  • patients with acute neurological status changes need an emergent evaluation of the airway status, breathing/oxygenation, and circulatory system (ABCs).
  • This scenario may occur in patients with acute hydrocephalus and may also be true for patients presenting with generalized tonic-clonic or subclinical seizure activity.
  • Rarely, patients may present with acute alteration in mental status and obtundation signifying acute hydrocephalus secondary to CSF outflow obstruction, which may occur with intraventricular tumors such as central neurocytoma or with seizure.
  • Its most useful feature is demonstrating hydrocephalus, which may require emergent surgical action.
  • Headache, vomiting, and papilledema are signs of elevated intracranial pressure, which is expected with space-occupying lesions.
  • Post-ictal patients and those presenting with acute status change secondary to acute hydrocephalus require frequent serial neurological evaluation.
  • The initial evaluation of a patient suspected of a neuronal or glioneuronal brain tumor requires a detailed history and physical examination
  • Patients presenting with acute deficit should first undergo a head CT without contrast after clinical stabilization
  • MRI is the imaging test of choice for evaluating this group of brain tumors.
  • Sequences should include T1—and T2-weighted images with and without gadolinium contrast.
  • Fluid-attenuated inversion recovery (FLAIR) imaging for tumor characterization and edema, susceptibility-weighted imaging (SWI) for hemorrhage and calcification, diffusion-weighted imaging (DWI) for an assessment of cellularity, and perfusion-weighted imaging (PWI) for an assessment of angiogenesis should be included as part of a standard brain tumor imaging protocol when a primary CNS tumor is suspected.
  • Other advanced MRI techniques, such as MR spectroscopy (MRS), diffusion tensor imaging (DTI), and functional MRI (fMRI), can also be useful.
  • An important consideration for this group of brain tumors is electroencephalography (EEG) since they commonly present with seizures.
  • Spot EEG may be useful in some cases, but continuous video EEG monitoring allows for better characterization of seizure semiology and assists with the localization of an epileptogenic focus.
  • Invasive EEG monitoring with either subdural strip electrodes or depth electrodes can also be considered in patients with seizures that cannot be localized to the region of the tumor with noninvasive recording techniques.
  • WADA testing is typically employed in these patients before resection to identify language dominance and assist with surgical planning.
  • LEAT patients should be discussed in interprofessional epilepsy conferences involving neurosurgeons, epilepsy neurologists, neuroradiologists, and neuropathologists to make appropriate diagnostic and treatment plans for these often-complicated patients.
  • Furthermore, these patients and others with tumors that are identified for another reason should be discussed at an interprofessional tumor board conference consisting of neurosurgeons, neurologists, neuroradiologists, neuropathologists, radiation oncologists, and medical oncologists to assist with appropriate diagnostic and treatment planning.
  • Asymptomatic or minimally symptomatic lesions identified on imaging for another reason can be reasonably followed with serial examination and neurological imaging due to the primarily low-grade nature of this group of tumors.
  • Medical treatment for this group of tumors chiefly encompasses the use of anti-epileptic medications for LEATs.
  • Surgical resection is the treatment of choice for symptomatic neuronal and glioneuronal tumors.
  • Patients who present with acute obstructive hydrocephalus require emergent surgery to prevent permanent neurological injury and death.
  • This typically involves a craniotomy with the goal of gross total tumor resection.
  • Gross total resection for higher-grade lesions appears to be more important.
  • Laser interstitial thermal therapy (LITT) is another emerging minimally invasive surgical strategy for intracranial lesions that has been applied to brain tumors and epilepsy surgery.
  • However, it may emerge as an alternative to formal surgical resection for tumors in deep-seated and eloquent tissues.
  • The role of radiation is somewhat limited for this group of tumors since most are WHO grade I.
  • However, higher-grade lesions including anaplastic ganglioglioma, liponeurocytoma, and some central/extraventricular neurocytoma may require adjuvant radiation therapy.
  • Chemo- and immuno-therapies are typically reserved for grade 2 and 3 tumors in a fashion similar to radiation therapy.
  • Immunotherapies targeting BRAF are emerging as adjuvant therapy for use in anaplastic ganglioglioma.
  • The use of chemotherapy in central neurocytoma is variable and typically reserved for patients with recurrent tumors.
  • Changes to the radiological characteristics of the tumor or the development of new neurological symptoms should prompt discussion at interprofessional conferences to consider intervention.
  • Adverse effects from anti-epileptic medications are common and include irritability, dizziness, drowsiness, blurred vision, difficulty with coordination, generalized fatigue, and weight gain.
  • More serious side effects are also possible, including severe skin reactions and congenital disabilities in children if taken during pregnancy.
  • Other complications are related to surgical intervention, including infection, intracranial and extracranial bleeding, postoperative hydrocephalus, new neurological deficit, lack of seizure control, injury to structures surrounding the tumor such as cranial nerves or blood vessels, cerebral edema and brain swelling, and death.
  • Patients with tumor-related epilepsy should be educated regarding seizure precautions and avoidance of secondary injury that may occur during a seizure.
  • Family members should also be educated in recognizing seizure activity and the necessary actions to take when a seizure occurs.
  • Patients with brain tumors should notify their provider of any new neurological symptoms, potentially representing changes to the tumor.
  • Emergency medical personnel must recognize acute neurological status changes to expedite emergency evaluation and medical or surgical responses, which helps minimize morbidity and mortality related to brain tumors.
  • review
    Neuronal Brain Tumors. StatPearls [Internet] — Frank M. Mezzacappa, William Thorell — StatPearls Publishing, 2022

Decision support only. Verify against local protocols and current labeling. Source: www.ncbi.nlm.nih.gov