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Synchronous multicentric pleomorphic xanthoastrocytoma: case report.

Sean A McNatt1, Ignacio Gonzalez-Gomez, Marvin D Nelson

  • 1Department of Neurosurgery, Keck School of Medicine, University of Southern California, Los Angeles, California 90033, USA. mcnatt@usc.edu

Neurosurgery
|July 1, 2005
PubMed
Summary

This study details a rare case of synchronous multicentric pleomorphic xanthoastrocytoma (PXA) in a pediatric patient. Treatment involved biopsy and radiation, with no disease progression observed after three years.

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Area of Science:

  • Pediatric neuro-oncology
  • Rare brain tumors
  • Astrocytoma research

Background:

  • Pleomorphic xanthoastrocytoma (PXA) is a rare, low-grade astrocytoma typically affecting adolescents.
  • Complete surgical resection generally yields favorable outcomes.
  • Limited data exist on treating recurrent, deep-seated, or multicentric PXA.

Observation:

  • A 13-year-old girl presented with headaches, polyuria, and fatigue, exhibiting papilledema, diabetes insipidus, and hypothyroidism.
  • MRI revealed numerous small, enhancing lesions scattered throughout both cerebral hemispheres.
  • Histological examination confirmed PXA.

Findings:

  • The patient underwent excisional biopsy and whole-brain radiation therapy with intensity-modulated boosts.

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  • Three years post-treatment, the patient remains neurologically stable with no evidence of disease progression.
  • Future surgical intervention is reserved for progressing lesions.

    • Implications:

    • Synchronous multicentric PXA poses therapeutic challenges due to potential surgical morbidity and the risk of anaplastic transformation.
    • This case highlights a potential treatment strategy for this rare presentation.
    • Further research is needed to establish optimal treatment protocols for multicentric PXA.