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.