Presentation of Supratentorial Pleomorphic Xanthoastrocytomas in Children

Symptoms

• Seizures most common symptom: Seizures are the most common presenting symptom, usually long-lasting seizures that were not investigated by imaging at their onset.
• Occasionally, symptoms due to elevated ICP: Less frequently, patients may develop intracranial hypertension.
• Focal neurological deficits unusual 

Patterns of evolution

• Seizures: Because seizures usually are the first presenting symptom, the tumor is most often diagnosed with imaging following seizures.

Time for evolution

• Slow: Usually PXAs are slow-growing tumors unless the tumor is an anaplastic variant.

Intervention at Presentation

Stabilization

• Seizure: Antiseizure medication is used to manage the presenting complaint.

Preparation for definitive intervention, nonemergent

• Imaging: MRI should be done.
• Plan surgery: After imaging, a surgical approach can be chosen and the case discussed with the parents. Surgery can be done as a semi-elective case in the OR.

Preparation for definitive intervention, emergent

• Manage hydrocephalus: In rare cases, if the tumor is large enough or causes hydrocephalus by blocking CSF pathways, then hydrocephalus has to be dealt with (i.e., EVD or direct removal of the tumor).
• Imaging: Imaging should be done. If the child’s condition is unstable, a CT scan will suffice to assess the pathology and the hydrocephalus.

Admission Orders

• Routine: Standard admission orders are used in preparing the child for surgery.