Intradural prepontine chordoma in an 11-year-old boy. A case report

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.