A paediatric supratentorial primitive neuroectodermal tumour associated with malignant astrocytic transformation and a clonal origin of both components.
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.
Kuhn
Susanne A
SA
Department of Neurosurgery, Medical Centre, Friedrich-Schiller-University, Erlanger Allee 101, 07747 Jena, Germany. Susanne.Kuhn@med.uni-jena.de
Hanisch
Uwe-Karsten
UK
Ebmeier
Kristian
K
Beetz
Christian
C
Brodhun
Michael
M
Reichart
Rupert
R
Ewald
Christian
C
Deufel
Thomas
T
Kalff
Rolf
R
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