title: A paediatric supratentorial primitive neuroectodermal tumour associated with malignant astrocytic transformation and a clonal origin of both components.
abstract: The case of a 7-year-old boy suffering from a supratentorial primitive neuroectodermal tumour (sPNET) at the age of 5 is presented. The tumour has been characterized by astrocytic areas within the sPNET revealing malignant transformation up to a multiform glioblastoma during the course of the disease. The clonal origin of both tumour components was established by loss of heterozygosity (LOH) analysis. Clinically, the tumour showed an aggressive biological behaviour with two recurrences. We discuss this very rare case and the first description of the clonal origin of distinct and distinguishable tumour components taking into consideration published literature.
authors.affiliation: Department of Neurosurgery, Medical Centre, Friedrich-Schiller-University, Erlanger Allee 101, 07747 Jena, Germany. Susanne.Kuhn@med.uni-jena.de
authors.forename: Susanne A
authors.initials: SA
authors.lastname: Kuhn
authors.forename: Uwe-Karsten
authors.initials: UK
authors.lastname: Hanisch
authors.forename: Kristian
authors.initials: K
authors.lastname: Ebmeier
authors.forename: Christian
authors.initials: C
authors.lastname: Beetz
authors.forename: Michael
authors.initials: M
authors.lastname: Brodhun
authors.forename: Rupert
authors.initials: R
authors.lastname: Reichart
authors.forename: Christian
authors.initials: C
authors.lastname: Ewald
authors.forename: Thomas
authors.initials: T
authors.lastname: Deufel
authors.forename: Rolf
authors.initials: R
authors.lastname: Kalff
mesh_terms: D002471:Cell Transformation, Neoplastic; D002648:Child; D002675:Child, Preschool; D005909:Glioblastoma / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000473:pathology* / Q000601:surgery; D018242:Neuroectodermal Tumors, Primitive / Q000473:pathology* / Q000601:surgery; D015173:Supratentorial Neoplasms / Q000473:pathology* / Q000601:surgery