Hdl Handle:
http://hdl.handle.net/10675.2/614
Title:
Pediatric primary intramedullary spinal cord glioblastoma
Authors:
Lober, Robert M.; Sharma, Suash; Bell, Beverly; Free, Alan; Figueroa, Ramon; Sheils, Chris W; Lee, Mark R.; Cowell, John K. ( 0000-0002-2079-5950 )
Abstract:
Spinal cord tumors in pediatric patients are rare, representing less than 1% of all central nervous system tumors. Two cases of pediatric primary intramedullary spinal cord glioblastoma at ages 14 and 8 years are reported. Both patients presented with rapid onset paraparesis and quadraparesis. Magnetic resonance imaging in both showed heterogeneously enhancing solitary mass lesions localized to lower cervical and upper thoracic spinal cord parenchyma. Histopathologic diagnosis was glioblastoma. Case #1 had a small cell component (primitive neuroectodermal tumor-like areas), higher Ki67, and p53 labeling indices, and a relatively stable karyotype with only minimal single copy losses involving regions: Chr8;pter-30480019, Chr16;pter-29754532, Chr16;56160245â 88668979, and Chr19;32848902-qter on retrospective comparative genomic hybridization using formalin-fixed, paraffin-embedded samples. Case #2 had relatively bland histomorphology and negligible p53 immunoreactivity. Both underwent multimodal therapy including gross total resection, postoperative radiation and chemotherapy. However, there was no significant improvement in neurological deficits, and overall survival in both cases was 14 months.This report highlights the broad histological spectrum and poor overall survival despite multi modality therapy. The finding of relatively unique genotypic abnormalities resembling pediatric embryonal tumors in one case may highlight the value of genome-wide profiling in development of effective therapy. The differences in management with intracranial and low-grade spinal cord gliomas and current management issues are discussed.; Key words
Citation:
Rare Tumors. 2010 Sep 30; 2(3):e48
Issue Date:
30-Sep-2010
URI:
http://hdl.handle.net/10675.2/614
DOI:
10.4081/rt.2010.e48
PubMed ID:
21139963
PubMed Central ID:
PMC2994522
Type:
Article
ISSN:
2036-3613
Appears in Collections:
Department of Pathology: Faculty Research and Presentations

Full metadata record

DC FieldValue Language
dc.contributor.authorLober, Robert M.en_US
dc.contributor.authorSharma, Suashen_US
dc.contributor.authorBell, Beverlyen_US
dc.contributor.authorFree, Alanen_US
dc.contributor.authorFigueroa, Ramonen_US
dc.contributor.authorSheils, Chris Wen_US
dc.contributor.authorLee, Mark R.en_US
dc.contributor.authorCowell, John K.en_US
dc.date.accessioned2012-10-26T16:26:53Z-
dc.date.available2012-10-26T16:26:53Z-
dc.date.issued2010-09-30en_US
dc.identifier.citationRare Tumors. 2010 Sep 30; 2(3):e48en_US
dc.identifier.issn2036-3613en_US
dc.identifier.pmid21139963en_US
dc.identifier.doi10.4081/rt.2010.e48en_US
dc.identifier.urihttp://hdl.handle.net/10675.2/614-
dc.description.abstractSpinal cord tumors in pediatric patients are rare, representing less than 1% of all central nervous system tumors. Two cases of pediatric primary intramedullary spinal cord glioblastoma at ages 14 and 8 years are reported. Both patients presented with rapid onset paraparesis and quadraparesis. Magnetic resonance imaging in both showed heterogeneously enhancing solitary mass lesions localized to lower cervical and upper thoracic spinal cord parenchyma. Histopathologic diagnosis was glioblastoma. Case #1 had a small cell component (primitive neuroectodermal tumor-like areas), higher Ki67, and p53 labeling indices, and a relatively stable karyotype with only minimal single copy losses involving regions: Chr8;pter-30480019, Chr16;pter-29754532, Chr16;56160245â 88668979, and Chr19;32848902-qter on retrospective comparative genomic hybridization using formalin-fixed, paraffin-embedded samples. Case #2 had relatively bland histomorphology and negligible p53 immunoreactivity. Both underwent multimodal therapy including gross total resection, postoperative radiation and chemotherapy. However, there was no significant improvement in neurological deficits, and overall survival in both cases was 14 months.This report highlights the broad histological spectrum and poor overall survival despite multi modality therapy. The finding of relatively unique genotypic abnormalities resembling pediatric embryonal tumors in one case may highlight the value of genome-wide profiling in development of effective therapy. The differences in management with intracranial and low-grade spinal cord gliomas and current management issues are discussed.en_US
dc.description.abstractKey wordsen_US
dc.rights©Copyright R. Lober et al., 2010en_US
dc.subjectCase Reporten_US
dc.titlePediatric primary intramedullary spinal cord glioblastomaen_US
dc.typeArticleen_US
dc.identifier.pmcidPMC2994522en_US
dc.contributor.corporatenameDepartment of Pathology-
dc.contributor.corporatenameDepartment of Pediatrics-
dc.contributor.corporatenameDepartment of Neurosurgery-
dc.contributor.corporatenameGHSU Cancer Center-

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