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A paediatric supratentorial primitive neuroectodermal tumour associated with malignant astrocytic transformation and a clonal origin of both components

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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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Acknowledgements

This work was supported by LUBOM Thuringia of the Ministry for Science, Research, and Art to SAK.

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Authors and Affiliations

  1. Department of Neurosurgery, Medical Centre, Friedrich-Schiller-University, Erlanger Allee 101, 07747, Jena, Germany

    Susanne A. Kuhn, Kristian Ebmeier, Rupert Reichart, Christian Ewald & Rolf Kalff

  2. Institute of Neuropathology, Medical Centre, Georg-August-University, Goettingen, Germany

    Uwe-Karsten Hanisch

  3. Clinical Chemistry, Research Centre, Friedrich-Schiller-University, Jena, Germany

    Christian Beetz & Thomas Deufel

  4. Institute of Pathology, Medical Centre, Friedrich-Schiller-University, Jena, Germany

    Michael Brodhun

Authors
  1. Susanne A. Kuhn
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  2. Uwe-Karsten Hanisch
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  3. Kristian Ebmeier
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  4. Christian Beetz
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  5. Michael Brodhun
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  6. Rupert Reichart
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  7. Christian Ewald
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Corresponding author

Correspondence to Susanne A. Kuhn.

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Comments

Axel Pagenstecher, Marburg, Germany

This is an interesting case report demonstrating the histopathological course of a supratentorial PNET (sPNET) in a five year old boy. The tumor was surgically removed and treated by irradiation and chemotherapy. Three months later, a low grade astrocytoma developed and in the histological specimens an astrocytic tumor with no detectable neuronal differentiation was observed. Thirteen months later, a glioblastoma was diagnosed. Analysis of LOH of the PNET and the low grade astrocytoma suggested a common clonal origin of both tumors. This case represents an unusual course of a PNET. Supratentorial PNETs often have a grave prognosis, with only about a third of the patients surviving 5 years. The tumors show a strong tendency to seed via the CSF. In the patient presented here, the occurrence of low grade astrocytoma with no PNET residues three months after first surgery is intriguing and raises the question whether there were more malignant portions of the tumor that evaded histological examination. The clinical course of the disease with extremely proliferating glioblastoma 13 months later supports the notion that highly malignant tumor cells remained in situ after the second surgical intervention.

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Kuhn, S.A., Hanisch, UK., Ebmeier, K. et al. A paediatric supratentorial primitive neuroectodermal tumour associated with malignant astrocytic transformation and a clonal origin of both components. Neurosurg Rev 30, 143–149 (2007). https://doi.org/10.1007/s10143-007-0069-5

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  • DOI: https://doi.org/10.1007/s10143-007-0069-5

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