title: Malignant progression of a pleomorphic xanthoastrocytoma in a child.
abstract: Pleomorphic xanthoastrocytoma (PXA) is a recently recognized rare cerebral neoplasm that predominantly affects young patients. We report on the case of a 3-year-old boy who presented with a 2-week history of headaches and seizures. Radiological investigation revealed a lesion in the right parietal-occipital lobe. The lesion was excised and histology disclosed the presence of a PXA with anaplastic features. 1 year later follow-up magnetic resonance imaging (MRI) revealed tumor relapse. An MRI of the spine was also performed and demonstrated leptomeningeal dissemination. The patient underwent a second operation. Histology revealed that the presence of a malignant PXA with anaplastic features. The patient received radiotherapy and 9 months later on follow-up MRI a new tumor recurrence was noted. A third craniotomy was performed and the tumor removed. Histological examination revealed dedifferentiation to glioblastoma multiforme. The patient was referred to the oncology department and received chemotherapy with temozolamide. 8 months later the patient was stable without tumor recurrence. PXAs require close follow-up because of their unpredictable biological behaviour.
authors.affiliation: Department of Neurosurgery, Children's Hospital "Agia Sofia", Athens, Greece. alexiougrg@yahoo.gr
authors.forename: G A
authors.initials: GA
authors.lastname: Alexiou
authors.forename: M
authors.initials: M
authors.lastname: Moschovi
authors.forename: K
authors.initials: K
authors.lastname: Stefanaki
authors.forename: C
authors.initials: C
authors.lastname: Prodromou
authors.forename: G
authors.initials: G
authors.lastname: Sfakianos
authors.forename: N
authors.initials: N
authors.lastname: Prodromou
mesh_terms: D001254:Astrocytoma / Q000150:complications / Q000175:diagnosis*; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000503:physiopathology*; D002675:Child, Preschool; D018450:Disease Progression; D005682:Gadolinium; D005909:Glioblastoma / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D014057:Tomography, X-Ray Computed / Q000379:methods
chemical_list: D005682:Gadolinium