A Paediatric Supratentorial Primitive Neuroectodermal Tumour Associated with Malignant Astrocytic Transformation and a Clonal Origin of Both Components

Overview

Journal Neurosurg Rev

Specialty Neurosurgery

Date 2007 Mar 3

PMID 17333087

Citations 2

Authors

Susanne A Kuhn

Uwe-Karsten Hanisch

Kristian Ebmeier

Christian Beetz

Michael Brodhun

Rupert Reichart

Christian Ewald

Thomas Deufel

Rolf Kalff

Affiliations

Soon will be listed here.

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.

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