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Clinico-radiologic characteristics of long-term survivors of diffuse intrinsic pontine glioma

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Abstract

Diffuse intrinsic pontine glioma (DIPG) is the deadliest central nervous system tumor in children. The survival of affected children has remained poor despite treatment with radiation therapy (RT) with or without chemotherapy. We reviewed the medical records of all surviving patients with DIPG treated at our institution between October 1, 1992 and May 31, 2011. Blinded central radiologic review of the magnetic resonance imaging at diagnosis of all surviving patients and 15 controls with DIPG was performed. All surviving patients underwent neurocognitive assessment during follow-up. Five (2.6 %) of 191 patients treated during the study period were surviving at a median of 9.3 years from their diagnosis (range 5.3–13.2 years). Two patients were younger than 3 years, one lacked signs of pontine cranial nerve involvement, and three had longer duration of symptoms at diagnosis. One patient had a radiologically atypical tumor and one had a tumor originating in the medulla. All five patients received RT. Chemotherapy was variable among these patients. Neurocognitive assessments were obtained after a median interval of 7.1 years. Three of four patients who underwent a detailed evaluation showed cognitive function in the borderline or mental retardation range. Two patients experienced disease progression at 8.8 and 13 years after diagnosis. A minority of children with DIPG experienced long-term survival with currently available therapies. These patients remained at high risk for tumor progression even after long follow-ups. Four of our long-term survivors had clinical and radiologic characteristics at diagnosis associated with improved outcome.

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Acknowledgments

This work was supported by the US National Institutes of Health Cancer Center Support (CORE) Grant P30 CA21765 and by the American Lebanese Syrian Associated Charities (ALSAC). All procedures described in this manuscript were performed in compliance with clinical research regulations in the United States.

Conflict of interest

The authors declare that they have no conflict of interest.

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Authors and Affiliations

  1. Department of Oncology, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Mailstop 260, Memphis, TN, 38105, USA

    Sadhana Jackson, Amar Gajjar & Alberto Broniscer

  2. Department of Radiological Sciences, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Memphis, TN, 38105, USA

    Zoltan Patay & Atmaram S. Pai Panandiker

  3. Department of Psychology, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Memphis, TN, 38105, USA

    Robyn Howarth

  4. Department of Biostatistics, St. Jude Children’s Research Hospital, 262 Danny Thomas Place, Memphis, TN, 38105, USA

    Arzu Onar-Thomas

  5. Department of Pediatrics, University of Tennessee Health Sciences Center, Memphis, TN, USA

    Amar Gajjar & Alberto Broniscer

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  1. Sadhana Jackson
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  2. Zoltan Patay
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  3. Robyn Howarth
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  4. Atmaram S. Pai Panandiker
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  6. Amar Gajjar
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  7. Alberto Broniscer
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Correspondence to Alberto Broniscer.

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Jackson, S., Patay, Z., Howarth, R. et al. Clinico-radiologic characteristics of long-term survivors of diffuse intrinsic pontine glioma. J Neurooncol 114, 339–344 (2013). https://doi.org/10.1007/s11060-013-1189-0

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  • DOI: https://doi.org/10.1007/s11060-013-1189-0

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