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Pleomorphic Xanthoastrocytoma with Anaplastic Features: Retrospective Case Series.
Martin J Rutkowski1, Taemin Oh1, Gulen G Niflioglu2
1Department of Neurological Surgery, Division of Neuropathology, University of California, San Francisco, California, USA.
World Neurosurgery
|August 2, 2016
Summary
Anaplastic pleomorphic xanthoastrocytoma (aPXA) indicates a poorer prognosis than typical PXA. Aggressive initial surgical resection is recommended, as adjuvant therapies show limited efficacy in preventing progression or dissemination.
Area of Science:
- Neuro-oncology
- Pathology
- Clinical Neurology
Background:
- Pleomorphic xanthoastrocytoma (PXA) is a rare glioma with generally favorable outcomes.
- A subset of PXA exhibits anaplastic features (aPXA), associated with significantly worse prognosis.
- Limited research exists on the clinical importance of anaplastic features in PXA.
Purpose of the Study:
- To investigate the clinicopathologic features and outcomes of anaplastic pleomorphic xanthoastrocytoma (aPXA).
- To evaluate the impact of treatment modalities on aPXA progression and survival.
Main Methods:
- Retrospective analysis of 8 patients diagnosed with aPXA between 1999-2012.
- Review of neuropathology for confirmation and WHO 2007 criteria adherence.
- Compilation of clinical data including demographics, tumor characteristics, treatment, and outcomes.
Main Results:
- The cohort included 5 females and 3 males, aged 4-74 years.
- Seizures were the most common symptom (50%); most tumors were in the frontal/temporal lobes (88%).
- Subtotal resection (STR) in 6 patients led to progression despite adjuvant therapy; median progression time was 20 months, 1-year progression-free survival was 57%. Median survival was 87 months, with 3 deaths. Four patients had disseminated disease. BRAFv600 mutation was present in 38%.
Conclusions:
- Anaplastic pleomorphic xanthoastrocytoma (aPXA) is linked to inferior clinical outcomes compared to typical PXA.
- Gross total resection is advised as the primary treatment goal.
- The efficacy of adjuvant radiation and chemotherapy in preventing progression/dissemination requires further study. Long-term patient monitoring is crucial due to potential for malignant transformation.
Keywords:
AnaplasticAstrocytomaGlioblastomaGliomaOutcomesPleomorphicSurgeryTreatmentXanthoastrocytoma
