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Unilateral adult malignant optic nerve glioma

  • Clinical Investigation
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Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

Abstract

Introduction

Adult malignant optic nerve gliomas are rare and rapidly fatal visual pathway tumours. They represent a clinical entity different from the more common childhood benign optic nerve gliomas, which are frequently associated with neurofibromatosis I.

Case report

A 61-year-old woman presented with rapidly progressing right vision loss, lower altitudinal visual field defect and papilloedema. MRI showed intraorbital and intracranial swelling of the right optic nerve. Resection of the intracranial part of the right optic nerve up to the chiasm revealed anaplastic astrocytoma grade III. Within 1 year, the patient died of leptomeningeal metastasis despite radiotherapy. Clinical and MRI evaluation of the left eye and optic nerve were normal at all times.

Discussion

Unilateral adult malignant glioma of the optic nerve is exceptional. The final diagnosis was only confirmed by optic nerve biopsy. In the literature, only one patient has been reported with a unilateral tumour manifestation; he was lost to follow-up 3 months later. All other cases were bilateral. To date, 44 case reports of adult malignant optic nerve glioma have been published, either malignant astrocytoma or glioblastoma. These tumours can mimic optic neuritis in their initial presentation. The diagnosis is seldom made before craniotomy. On MRI images, malignant glioma cannot be distinguished from optic nerve enlargement due to other causes. Although radiotherapy appears to prolong life expectancy, all presently available treatment options (radiation, surgery, radio-chemotherapy) are of limited value. Most patients go blind and die within 1 or 2 years.

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Acknowledgements

The authors would like to thank C. Sing for the English revision.

Author information

Authors and Affiliations

  1. Department of Paediatric Ophthalmology, Strabismology and Ophthalmogenetics, University of Regensburg, Franz Josef Strauss Allee 11, 93053, Regensburg, Germany

    Bettina Wabbels, Anke Demmler & Birgit Lorenz

  2. Institute of Radiology, University of Regensburg, Regensburg, Germany

    Johannes Seitz

  3. Institute of Pathology, University of Regensburg, Regensburg, Germany

    Matthias Woenckhaus

  4. Department of Neurosurgery, University of Regensburg, Regensburg, Germany

    Heinz-Georg Bloß

Authors
  1. Bettina Wabbels
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  2. Anke Demmler
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  3. Johannes Seitz
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  4. Matthias Woenckhaus
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  5. Heinz-Georg Bloß
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  6. Birgit Lorenz
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Corresponding author

Correspondence to Bettina Wabbels.

Additional information

This paper was presented in part at the 101st Meeting of the German Ophthalmological Society (DOG).

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Wabbels, B., Demmler, A., Seitz, J. et al. Unilateral adult malignant optic nerve glioma. Graefe's Arch Clin Exp Ophthalmol 242, 741–748 (2004). https://doi.org/10.1007/s00417-004-0905-z

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  • DOI: https://doi.org/10.1007/s00417-004-0905-z

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