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Primary gliomatosis cerebri involving gray matter in pediatrics: a distinct entity? A multicenter study of 14 cases

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Abstract

Background and purpose

Gliomatosis cerebri (GC) is a rare neoplasm including a variety of tumors, with extremely variable evolution and heterogeneity of prognosis. It may appear either de novo or after a focal glioma, involve predominantly the white or the gray matter, and concern either pediatric or adult patients. We focused on primary GC involving exclusively gray matter in a pediatric population in order better to define the presentation and outcome of this disease.

Patients and methods

We reviewed the databases of seven Departments of Pediatric Oncology to identify pediatric cases of GC between 1990 and 2007. Patients were included if they demonstrated a diffuse infiltrative process involving gray matter in magnetic resonance imaging (MRI) and histological tissue analyses, confirming a proliferative glial disorder.

Results

Fourteen patients with a median age of 8 years were identified. Epilepsy was the main presenting symptom. Brain MRI showed a lesion of the temporal and insular cerebral cortex associated with tumoral infiltration of the thalami and the basal ganglia. Histological examination confirmed the diagnosis of high-grade glioma. Prognosis was always very gloomy in the short term, with a median survival of less than a year.

Conclusion

This rare entity, whose prognosis is appalling whatever the treatment proposed, should be clearly identified within the heterogeneous group of GC in the same way as diffuse intrinsic pontine gliomas have been identified among brain stem tumors. Systematic biopsies appear essential to permit the molecular studies which will assist in guiding the choice of future targeted treatments.

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Acknowledgments

The authors thank Mrs. Deirdre McKeown for her help with the English language

Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Pediatric Oncology, Pontchaillou University Hospital, Rennes, France

    Céline Chappé & Christine Edan

  2. Department of Pediatric Neurosurgery, Rennes University Hospital, Rennes, France

    Laurent Riffaud

  3. Department of Neuroradiology, Pontchaillou University Hospital, Rennes, France

    Catherine Tréguier & Béatrice Carsin-Nicol

  4. Department of Epidemiology and Public Health, Pontchaillou University Hospital, Rennes, France

    David Veillard

  5. Department of Neuropathology, Pontchaillou University Hospital, Rennes, France

    Dan Cristian Chiforeanu

  6. Department of Pediatric Oncology, Pontchaillou University Hospital, Villejuif, France

    Jacques Grill

  7. Department of Pediatric Oncology, Pontchaillou University Hospital, Lyons, France

    Didier Frappaz

  8. Department of Pediatric Oncology, Pontchaillou University Hospital, Marseilles, France

    Nicolas André

  9. Department of Pediatric Oncology, Pontchaillou University Hospital, Poitiers, France

    Fréderic Millot

  10. Department of Pediatric Oncology, Pontchaillou University Hospital, Nice, France

    Nicolas Sirvent

  11. Department of Pediatric Neurosurgery, Pontchaillou University Hospital, Lille, France

    Matthieu Vinchon

Authors
  1. Céline Chappé
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  2. Laurent Riffaud
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  3. Catherine Tréguier
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  4. Béatrice Carsin-Nicol
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  5. David Veillard
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  6. Dan Cristian Chiforeanu
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  7. Jacques Grill
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  8. Didier Frappaz
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  9. Nicolas André
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  10. Fréderic Millot
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  11. Matthieu Vinchon
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  12. Nicolas Sirvent
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  13. Christine Edan
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Corresponding author

Correspondence to Laurent Riffaud.

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Chappé, C., Riffaud, L., Tréguier, C. et al. Primary gliomatosis cerebri involving gray matter in pediatrics: a distinct entity? A multicenter study of 14 cases. Childs Nerv Syst 29, 565–571 (2013). https://doi.org/10.1007/s00381-012-2016-1

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  • DOI: https://doi.org/10.1007/s00381-012-2016-1

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