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Intramedullary spinal cord primitive neuroectodermal tumor presenting with hydrocephalus
George A Alexiou1, George Siozos, Kalliopi Stefanaki
1Department of Neurosurgery, Children's Hospital Agia Sofia, Athens, Greece. alexiougrg@yahoo.gr
Journal of Child Neurology
|April 26, 2012
Summary
This case study details an extremely rare spinal primitive neuroectodermal tumor in an infant, presenting with hydrocephalus and hemiparesis. Further multi-institutional research is crucial for establishing treatment guidelines for these rare central nervous system tumors.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Spinal Cord Tumors
Background:
- Spinal primitive neuroectodermal tumors (SPNETs) are exceptionally rare central nervous system (CNS) neoplasms.
- Intramedullary spinal cord tumors are uncommon in pediatric populations.
Observation:
- A 2-month-old boy presented with left hemiparesis and a prenatal diagnosis of an enlarged ventricular system.
- Magnetic resonance imaging (MRI) revealed an intramedullary lesion from C2 to T1, associated with hydrocephalus and intracranial/spinal dissemination.
- The tumor was diagnosed as a CNS primitive neuroectodermal tumor (WHO grade IV).
Findings:
- This is the first reported case of an intramedullary spinal primitive neuroectodermal tumor associated with hydrocephalus.
- Surgical resection was performed after ventriculoperitoneal shunt placement, with some residual tumor.
- No postoperative neurological deterioration was observed.
Implications:
- The extreme rarity of spinal primitive neuroectodermal tumors necessitates multi-institutional studies to develop effective treatment guidelines.
- Early diagnosis and management of associated hydrocephalus are critical in pediatric cases.
- Further research is needed to understand the unique characteristics and optimal treatment strategies for these rare tumors.
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