Juvenile intradural chordoma: case report.

Neurosurgery

Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, AZ 85013, USA.

Published: February 2008

Objective: We report the youngest known case of a prepontine intradural chordoma. These tumors are exceedingly rare. Unlike their more common extradural counterparts, no recurrence of an intradural chordoma has been reported.

Clinical Presentation: A 9-year-old boy underwent diagnostic imaging for evaluation of headaches. Although neurologically intact, a magnetic resonance imaging scan revealed a large prepontine mass with focal enhancement.

Intervention: Endoscopic-assisted gross total resection was attained with staged bilateral retrosigmoid approaches. There were no additional adjuvant therapies. At the time of the 1-year follow-up evaluation, the patient had no recurrence.

Conclusion: By using an endoscopic-assisted procedure, we achieved complete resection of an intradural chordoma offering a potential for surgical cure. Resection is particularly advantageous because it spares the young child the need for radiation treatment. Close follow-up is warranted because we postulate that this tumor exists in a biological continuum between benign notochordal hamartomatous remnants and typical invasive chordomas.

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Source
http://dx.doi.org/10.1227/01.neu.0000316022.74162.00DOI Listing

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