title: Intradural prepontine chordoma in an 11-year-old boy. A case report.
abstract: METHODS
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurosurgery, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands. Saman.Vinke@radboudumc.nl.
authors.forename: R Saman
authors.identifier: http://orcid.org/0000-0003-3971-1849
authors.initials: RS
authors.lastname: Vinke
authors.affiliation: Department of Neurosurgery, St. Elisabeth Hospital, Tilburg, The Netherlands.
authors.forename: Elise Charlotte
authors.initials: EC
authors.lastname: Lamers
authors.affiliation: Department of Pathology, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Benno
authors.initials: B
authors.lastname: Kusters
authors.affiliation: Department of Neurosurgery, Radboud University Medical Center, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands.
authors.forename: Erik J
authors.initials: EJ
authors.lastname: van Lindert
mesh_terms: D001932:Brain Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D002648:Child; D002817:Chordoma / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D014057:Tomography, X-Ray Computed; D017287:Ventriculoperitoneal Shunt / Q000379:methods*
keywords: Chordoma; Intradural; Paediatric; Prepontine