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Prospective neuraxis MRI surveillance reveals a high risk of leptomeningeal dissemination in diffuse intrinsic pontine glioma

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Abstract

Prognosis of diffuse intrinsic pontine gliomas (DIPGs) remains poor. Failure has been predominantly local, with leptomeningeal dissemination (LD) occurring in 4–33% of patients in pre-MRI era series. Routine craniospinal imaging after initial treatment may reveal other relapse patterns relapse. Sixteen consecutive pediatric patients with DIPG treated between 2006 and 2009 were retrospectively reviewed. Treatment regimens, recurrence patterns, survival, and pathologic diagnosis were recorded. Fourteen patients received involved-field radiotherapy to 54 Gy, and two patients received craniospinal irradiation for LD at presentation. Neuraxis MRI was performed at diagnosis and at 4 month intervals following radiotherapy. Fifteen patients have had progression of disease (median progression-free survival 5.0 ± 1.2 months), and 13 patients have died (median survival 9.0 ± 1.4 months). Local failure occurred in 12 patients (75%). LD occurred in nine patients (56%). LD was present at diagnosis in three patients, after initial staging and treatment in six patients, and during autopsy in two patients. Median overall survival was 12.0 ± 3.3 months without LD and 8.0 ± 2.1 months with LD (P = 0.059, log rank test). Median progression-free survival was 9.5 ± 3.9 months without LD and 3.0 ± 2.1 months with LD (P = 0.012, log rank test). The high incidence of LD probably reflects liberal use of spine MRI surveillance. All patients should undergo routine craniospinal imaging at diagnosis and follow-up. Central nervous system prophylaxis should be considered in future clinical trials.

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Authors and Affiliations

  1. Department of Radiation Oncology, New York University Langone Medical Center, 566 First Avenue, New York, NY, 10014, USA

    Rajni Sethi, Bernadine Donahue, Saroj Kunnakkat & Ashwatha Narayana

  2. Department of Pediatrics, New York University Langone Medical Center, New York, NY, USA

    Jeffrey Allen, Matthias Karajannis, Sharon Gardner & Jeena Mathew

  3. Department of Neurology, New York University Langone Medical Center, New York, NY, USA

    Jeffrey Allen

  4. Department of Neurosurgery, New York University Langone Medical Center, New York, NY, USA

    Jeffrey Wisoff & Ashwatha Narayana

  5. Department of Pathology, New York University Langone Medical Center, New York, NY, USA

    David Zagzag & Kia Newman

  6. Department of Radiation Oncology, Maimonides Medical Center, Brooklyn, NY, USA

    Bernadine Donahue

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  1. Rajni Sethi
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Sethi, R., Allen, J., Donahue, B. et al. Prospective neuraxis MRI surveillance reveals a high risk of leptomeningeal dissemination in diffuse intrinsic pontine glioma. J Neurooncol 102, 121–127 (2011). https://doi.org/10.1007/s11060-010-0301-y

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