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Temporal pleomorphic xanthoastrocytoma with glycogen accumulation--case report
F Rogério1, L de Souza Queiroz, M S X de Lima
1Department of Pathology, School of Medical Sciences, State University of Campinas, UNICAMP, Brazil. fabio@fcm.unicamp.br
Clinical Neuropathology
|August 1, 2008
Summary
Pleomorphic xanthoastrocytoma (PXA) is a rare glioma in young patients. This case highlights unusual glycogen accumulation within tumor cells, offering new insights into glial tumor metabolism.
Area of Science:
- Neuro-oncology
- Cellular pathology
Background:
- Pleomorphic xanthoastrocytoma (PXA) is a rare, typically benign glioma predominantly affecting young individuals.
- PXA often presents as a contrast-enhancing lesion on imaging and is associated with a history of seizures.
Observation:
- A unique PXA case exhibited abundant intracytoplasmic glycogen deposits.
- Histochemical (PAS-positive, diastase-sensitive) and ultrastructural analysis confirmed glycogen accumulation.
- Tumor cells showed immunoreactivity for GFAP, vimentin, CD34, and EGFR, with minimal p53 expression.
Findings:
- The presence of extensive glycogen in PXA cells is an uncommon finding in glial tumors.
- CD34 positivity on the external membrane suggests a link to chronic CNS lesions, potentially epilepsy-related.
- EGFR overexpression may contribute to neoplastic proliferation, while limited p53 suggests it's not a primary driver.
Implications:
- This case expands the understanding of metabolic abnormalities in glial tumor development.
- Further research into carbohydrate metabolism pathways could reveal novel therapeutic targets for PXA.
- The findings contribute to the differential diagnosis and understanding of rare glial tumor subtypes.
