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Polar Spongioblastoma of the Spinal Cord: A Case Report

Authors :
Maryam Fouladi
Peter C. Burger
Kathleen J. Helton
Christine E. Fuller
Larry E. Kun
James T. Dalton
Source :
Pediatric and Developmental Pathology. 9:75-80
Publication Year :
2006
Publisher :
SAGE Publications, 2006.

Abstract

“Rhythmic palisading” is a striking histologic pattern infrequently encountered in a variety of central nervous system (CNS) tumors. We present the case of an infant with a large spinal cord lesion wherein all sampled tissue showed columnar arrangements of palisaded cells, typical of polar spongioblastoma. The tumor was briskly proliferative, focally necrotic, and variably expressed S100, glial fibrillary acidic protein, neuron specific enolase, and p53 by immunohistochemistry. Fluorescence in situ hybridization failed to reveal isochromosome 17q, EGFR amplification, or deletions of 1p, 19q, 22q11.2, 10q, or p16. Despite chemotherapy and decadron, he developed lesional necrosis and intracranial metastases and died less than 1 mo from presentation. This case illustrates polar spongioblastoma as a distinctive histologic pattern that can occur in embryonal CNS tumors. Discrimination of these rare aggressive lesions from other CNS tumors with focal palisaded architecture is crucial as the treatment and prognosis of the latter may differ significantly.

Details

ISSN :
16155742 and 10935266
Volume :
9
Database :
OpenAIRE
Journal :
Pediatric and Developmental Pathology
Accession number :
edsair.doi.dedup.....909cb45dc5ff4ec195c17c71c5d7571c
Full Text :
https://doi.org/10.2350/08-05-0099.1