title: A five year-old child with clear cell petro-clival meningioma: case report with clinical and histopathological long-term follow-up.
abstract: BACKGROUND
  Only a few cases have been previously published about clear cell meningiomas in children, the majority of them in the location of the spine. We describe an unusual case of clear cell meningioma occurring at the petro-clival region in a 5-year-old child. We further seek to determine the impact of several growth factors as well as the AKT1 mutation on the tumor growth pattern.
  
  
  METHODS
  A five-year-old girl was presented with a one-week history of cephalgia, ataxia, and left sided torticollis. Magnetic resonance imaging (MRI) revealed a dumbbell-shaped homogeneously petro-clival gadolinium-enhancing mass. A staged operative approach was chosen, and a complete removal of the tumor was achieved. Due to recurrent tumor progression, the child underwent several tumor surgeries and two cranial radiations. None of the treatments were able to stop tumor progression. Consequently, the child died at the age of 14 after further extensive intracranial and extracranial tumor progression. The initial histological examination revealed a clear cell meningioma WHO grade II with an MIB-1 labeling index of <1%, which gradually increased with every recurrence up to 10% by the last progression at the age of 13 years. Analogically, an increasing overexpression of epidermal growth factor receptor (EGFR), the platelet-derived growth factor receptor (PDGFR), and the vascular endothelial growth factor receptor (VEGFR) was observed with each recurrence. The AKT1 (E17K) mutation in the tumor was not detectable in all investigated specimens.
  
  
  CONCLUSIONS
  Pediatric clear cell meningiomas WHO grade II are very rare. Our data demonstrate the progressive overexpression of EGF-, PDGF-, and VEGF-receptors in each recurrence, providing one of these receptors as targeted therapy in such cases. Further evaluation of these growth factors in clear cell meningioma is required to establish the optimal treatment of these aggressive tumors.
authors.affiliation: Klinik und Poliklinik für Neurochirurgie, Medizinische Fakultät Carl Gustav Carus, An der Technische Universität Dresden, Fetscherstrasse 74, 01307, Dresden, Germany. tareq.juratli@uniklinikum-dresden.de.
authors.forename: Tareq A
authors.initials: TA
authors.lastname: Juratli
authors.affiliation: Institut für Pathologie, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
authors.forename: Kathrin D
authors.initials: KD
authors.lastname: Geiger
authors.affiliation: Klinik und Poliklinik für Neurochirurgie, Medizinische Fakultät Carl Gustav Carus, An der Technische Universität Dresden, Fetscherstrasse 74, 01307, Dresden, Germany.
authors.forename: Patrick
authors.initials: P
authors.lastname: Weigel
authors.affiliation: Abteilung Neuropädiatrie, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
authors.forename: Maja
authors.initials: M
authors.lastname: von der Hagen
authors.affiliation: Abteilung für Neuroradiologie, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
authors.forename: Dirk
authors.initials: D
authors.lastname: Daubner
authors.affiliation: Klinik und Poliklinik für Neurochirurgie, Medizinische Fakultät Carl Gustav Carus, An der Technische Universität Dresden, Fetscherstrasse 74, 01307, Dresden, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Pinzer
authors.affiliation: Institut und Poliklinik für Radiologische Diagnostik, Technische Universität Dresden, Dresden, Germany.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Hahn
authors.affiliation: Klinik und Poliklinik für Neurochirurgie, Medizinische Fakultät Carl Gustav Carus, An der Technische Universität Dresden, Fetscherstrasse 74, 01307, Dresden, Germany.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Schackert
authors.affiliation: Klinik und Poliklinik für Neurochirurgie, Medizinische Fakultät Carl Gustav Carus, An der Technische Universität Dresden, Fetscherstrasse 74, 01307, Dresden, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Kirsch
mesh_terms: D001921:Brain / Q000378:metabolism / Q000473:pathology*; D002675:Child, Preschool; D066246:ErbB Receptors; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008577:Meningeal Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D008579:Meningioma / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D009154:Mutation / Q000235:genetics; D051057:Proto-Oncogene Proteins c-akt / Q000235:genetics; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000378:metabolism; D040262:Receptors, Vascular Endothelial Growth Factor / Q000378:metabolism
chemical_list: D066246:ErbB Receptors; D020796:Receptor, Platelet-Derived Growth Factor alpha; D040262:Receptors, Vascular Endothelial Growth Factor; C494918:AKT1 protein, human; D051057:Proto-Oncogene Proteins c-akt
keywords: AKT1 mutation; Child; Clear cell meningioma; Epidermal growth factor receptor; Platelet-derived growth factor receptor; Vascular endothelial growth factor receptor