De Novo Malignant Optic Chiasm Glioma: Initial Clinical Response to Steroids

Abstract

Malignant optic nerve glioma is a rare but uniformly fatal disease that remains poorly understood. The authors describe a notable case of this rare disease occurring in the optic chiasm. Normal brain imaging and normal ophthalmic examination 2 years prior to diagnosis provide evidence for de novo genesis of malignant optic nerve glioma in their patient. The patient’s early response to steroids highlights the degree to which malignant optic nerve glioma can initially mimic inflammatory optic neuropathies and chiasmal syndromes. This case also demonstrates a poor outcome for malignant optic nerve glioma even with current advanced therapy for glioblastoma using radiotherapy plus concomitant and adjuvant temozolomide and bevacizumab.

Keywords::

• brain neoplasm
• inflammatory optic neuropathy
• malignant optic glioma
• optic chiasm

ACKNOWLEDGEMENTS

Dr. Moss receives support from the Laura K. Binder endowed fund (University of Illinois Department of Ophthalmology), and K12EY021475 (National Eye Institute).

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

Note: Figure 3 of this article is available in colour online at www.informahealthcare.com/oph