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Thoracic intramedullary chordoma without bone involvement: a rare clinical entity
Mohd Faheem1, Qazi Zeeshan1, Balkrishna Ojha1
1Department of Neurosurgery, King George's Medical University, Lucknow, Uttar Pradesh, India.
BMJ Case Reports
|July 30, 2016
Summary
This case study reports a rare pediatric intramedullary chordoma, a spinal cord tumor without bone involvement. The 8-year-old patient experienced significant clinical improvement after diagnosis and treatment.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Oncology
Background:
- Intramedullary spinal cord tumors are rare in children.
- Chordomas are typically bone tumors, with intramedullary chordomas being exceptionally uncommon.
- This case highlights a rare presentation of a spinal tumor in a pediatric patient.
Observation:
- An 8-year-old boy presented with a year of low back pain, paraparesis, and urinary incontinence.
- Spinal MRI revealed an intramedullary lesion from T11 to L1, initially suspected to be astrocytoma, ependymoma, or hemangioblastoma.
- Tissue biopsy and immunohistochemistry confirmed intramedullary chordoma.
Findings:
- This represents the second reported case of an intramedullary chordoma lacking bone involvement in the English literature.
- The diagnosis was confirmed via tissue biopsy and immunohistochemical analysis.
- The tumor was located within the spinal cord itself, extending from the T11 to L1 vertebral levels.
Implications:
- This case expands the known spectrum of pediatric spinal tumors.
- Early diagnosis and appropriate management of rare spinal neoplasms are crucial for pediatric outcomes.
- Further research into the pathogenesis and treatment of intramedullary chordomas is warranted.
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