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Malignant optic glioma – the spectrum of disease in a case series

  • Neurophthalmology
  • Published:
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Abstract

Purpose

Malignant optic glioma of adulthood is a rare, invasive neoplasm of the anterior visual pathway with 66 cases reported in the literature. It presents as anaplastic astrocytoma (WHO grade III) or glioblastoma (WHO grade IV). The present case series covers the spectrum of disease manifestations, discusses neuroradiological findings, and reviews the current literature.

Methods

Retrospective case series of five patients from three tertiary referral centers and literature review.

Results

Visual loss with or without pain was the presenting symptom in all patients (two women, three men). Two patients were initially misdiagnosed as optic neuritis, and one patient as atypical non-arteritic anterior ischemic optic neuropathy (NAION). A neoplastic disease was suspected in the two remaining patients. MRI features were iso- to hypointensity on T1-weighted native images, contrast enhancement, and hyperintensity on T2-weighted images. Biopsy was generally diagnostic; however, one patient required two biopsies for diagnosis. The series includes an exceptional case of intraocular tumor extension and vitreous spread. The disease was lethal within one to two years in all patients.

Conclusions

Malignant optic glioma is a diagnostic challenge and remains a devastating and lethal disease. Advances in the understanding of tumor biology have yet failed to translate into effective treatment regimens.

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Author information

Authors and Affiliations

  1. Department of Ophthalmology, University Hospital Zurich, Frauenklinikstrasse 24, CH-8091, Zurich, Switzerland

    Ghislaine L. Traber & Klara Landau

  2. Institute of Neuroradiology, University Hospital Zurich, Zurich, Switzerland

    Athina Pangalu

  3. Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland

    Manuela Neumann

  4. Department of Neurology, University Hospital Zurich, Zurich, Switzerland

    Michael Weller

  5. Department of Neuropathology, University of Tübingen, Tübingen, Germany

    Manuela Neumann

  6. Department of Ophthalmology, Egas Moniz Hospital, Lisbon, Portugal

    Joao Costa

  7. Goldschleger Eye Institute, Sheba Medical Center, Sackler School of Medicine Tel Aviv University, Tel Aviv-Yafo, Israel

    Ruth Huna-Baron

Authors
  1. Ghislaine L. Traber
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  2. Athina Pangalu
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  3. Manuela Neumann
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  4. Joao Costa
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  5. Michael Weller
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  6. Ruth Huna-Baron
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  7. Klara Landau
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Correspondence to Ghislaine L. Traber.

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Traber, G.L., Pangalu, A., Neumann, M. et al. Malignant optic glioma – the spectrum of disease in a case series. Graefes Arch Clin Exp Ophthalmol 253, 1187–1194 (2015). https://doi.org/10.1007/s00417-015-3045-8

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  • DOI: https://doi.org/10.1007/s00417-015-3045-8

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