Presentation of Supratentorial Central Neurocytomas in Children

Symptoms

• Elevated ICP: The majority of patients present with symptoms of elevated ICP. The mean clinical history is 3.5 months. Occasionally, visual and mental disturbances may be apparent.
• Seizures: Rarely, the patient may present with seizures.
• Hormonal abnormalities: Patients with neurocytomas of the septum pellucidum and hypothalamic region may present with hormonal disturbance.
• Intraventricular hemorrhage: Massive intraventricular hemorrhage has rarely been described as the initial presentation.

Patterns of evolution

• Headaches: Most patients with central neurocytomas present with headaches.
• Atypical neurocytomas aggressive: The atypical neurocytomas, which start in the same way, have a much more aggressive course, often presenting with marked neurological deficit.

Time for evolution

• 2–3 months: Symptoms evolve over 2–3 months.

Intervention

Since most central neurocytomas are benign tumors, complete surgical removal is always planned as the treatment of choice.

Stabilization

• Usually not required: Since these patients rarely present in an acute stage, no specific preparation is required for surgery. Usual preparations include IV dexamethasone, anticonvulsants if indicated, and standard anesthetic evaluations.

Preparation for definitive intervention, nonemergent

• Prepare for surgery: The patient needs to be prepared for surgical intervention, which is the treatment of choice. The planning for the choice of approach depends on the location of the tumor and the comfort level of the surgeon.

Preparation for definitive intervention, emergent

• Manage hydrocephalus: In case of acute hydrocephalus, insertion of an EVD may be necessary prior to definitive intervention. Patients may need anti-edema measures started.

Admission Orders

• CSF drainage parameters: In situations where the patients present with acute hydrocephalus and are unfit for definitive craniotomy, instructions regarding placement of an initial EVD need to be given.