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Intradural prepontine chordoma in an 11-year-old boy. A case report
R Saman Vinke1, Elise Charlotte Lamers2, Benno Kusters3
1Department of Neurosurgery, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands. Saman.Vinke@radboudumc.nl.
Insights
A rare pediatric intradural chordoma, a tumor from notochordal remnants, was successfully resected in an 11-year-old boy. He remained disease-free for six years post-surgery.
Area of Science:
- Pediatric Neurosurgery
- Neuro-oncology
- Chordoma Research
Background:
- Intradural chordomas are exceptionally rare pediatric tumors originating from notochordal remnants.
- Distinguishing intradural chordomas from ecchordosis physaliphora (EP) can be challenging due to similar biological behavior.
Observation:
- An 11-year-old boy presented with chronic headache and vomiting.
- Imaging revealed a large prepontine mass causing obstructive hydrocephalus.
- Surgical resection via a left frontotemporal transsylvian approach achieved total tumor removal.
Findings:
- Histological examination confirmed the diagnosis of chordoma.
- The tumor did not involve the dura or clivus.
- The patient experienced no recurrence or disease progression over a 6-year follow-up period.
Implications:
- Surgical resection is the recommended primary treatment for pediatric intradural chordomas.
- Adjuvant radiation therapy may not be necessary in pediatric cases.
- Further research into the continuum between benign notochordal tumors and malignant chordomas is warranted.
Case Report:
The authors report a case of an 11-year-old boy that presented with headache and vomiting that was present for several months. CT and MR imaging revealed a large prepontine mass and an obstructive hydrocephalus. A ventriculoperitoneal shunt was inserted, and in a second operation, a radiologically proven total resection was performed, using a left frontotemporal transsylvian approach. The tumour showed no involvement of the dura or clivus. Histological examination showed the characteristics of a chordoma. No further adjuvant treatment was given. The patient remained disease or tumour free after a 6-year follow-up.
Discussion:
Intradural chordomas are extremely rare tumours that originate from notochordal remnants. Only three other cases have been reported in the paediatric population. Ecchordosis physaliphora (EP) is an ectopic notochordal remnant that has a similar biological behaviour and is difficult to distinguish from intradural chordomas. They might exist in a continuum from benign notochordal tumour to malignant chordoma. A surgical resection without adjuvant radiation therapy is suggested to be the treatment of choice in the paediatric population.
Conclusion:
The authors describe a rare case of an intradural prepontine chordoma in an 11-year-old boy that stayed disease free after a 6-year follow-up.
