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Published on: March 7, 2017
Pediatric intracranial primary anaplastic ganglioglioma
Wolf Lüdemann1, Rouzbeh Banan2, Christian Hartmann2
1Neurosurgical Department, Helios Klinik Hildesheim, 31135, Hildesheim, Germany. Wolf.Luedemann@helios-kliniken.de.
Insights
Anaplastic gangliogliomas are rare pediatric brain tumors. This case highlights an axial tumor in an 11-year-old, emphasizing the need for prompt diagnosis and multimodal treatment.
Area of Science:
- Pediatric neuro-oncology
- Neuroradiology
- Surgical neurology
Background:
- Primary intracranial anaplastic gangliogliomas are rare in children, often presenting with increased intracranial pressure or epilepsy.
- These tumors typically occur in an extraaxial location and can mimic pilocytic astrocytomas on MRI.
- Surgical resection extent and adjuvant therapy are crucial for preventing recurrence.
Observation:
- An 11-year-old boy presented with headache and double vision due to obstructive hydrocephalus.
- Brain MRI revealed an axial, contrast-enhancing lesion in the quadrigeminal plate region, extending from the cerebellum to the pineal gland.
- The lesion caused significant hydrocephalus, necessitating intervention.
Findings:
- Subtotal surgical removal of the lesion was performed.
- Histopathological examination confirmed the diagnosis of an anaplastic ganglioglioma.
- The diagnosis was further validated by a reference center.
Implications:
- Early diagnosis and aggressive surgical management are vital for pediatric brain tumors.
- Adjuvant radiation therapy and chemotherapy are indicated for anaplastic gangliogliomas to improve outcomes.
- This case underscores the importance of considering axial lesions in the differential diagnosis of pediatric brain tumors.
Background:
Primary intracranial anaplastic gangliogliomas are rare tumors in the pediatric patient group. Most of them present with symptoms of elevated pressure or symptomatic epilepsy. Extraaxial location is far more common than axial location. On MRI examination, they mimic pilocytic astrocytomas. The outcome after surgery depends mainly on the possible amount of surgical resection, and oncological therapy is necessary to prevent recurrence of the disease.
Case Report:
An 11-year-old boy presented with headache and double vision due to obstructive hydrocephalus. MRI of the brain revealed an axial partially contrast enhancing lesion in the quadrigeminal plate extending from the cerebellum to the pineal gland and causing hydrocephalus. Subtotal removal of the lesion was performed, and the diagnosis of an anaplastic ganglioglioma was established and confirmed by the reference center. At the latest follow up (3 months), the boy is without any neurological symptoms and scheduled for radiation therapy as well as chemotherapy.

