Learning objectives
To present optic pathway glioma in adulthood and to describe imaging modalities of this disease with pictorial examples of the tumor and its differential diagnoses.
Background
Optic nerve gliomas are the most frequent neoplasms of the optic nerve. They account for 1% of all intracranial tumors (1). Only 25% of optic gliomas are confined to the optic nerve itself, while in 40-75% of cases optic gliomas involve chiasm, hypothalamus and other structures (2).
They mostly arise as histologically benign tumors during childhood and in young adults. However, a highly aggressive and malignant form of optic glioma can occur in older adults, which often results in blindness and fatal outcome in a...
Findings and procedure details
Optic glioma in childhood
The majority of patients with optic nerve glioma present before the age of 10 (1). Childhood cases are often seen in patients with neurofibromatosis type 1 (NF-1) (2). Optic nerve gliomas that arise in childhood are usually relatively benign, low-grade tumors, such as pilocytic or fibrillary astrocytoma (3).
Patients with optic nerve gliomas can be asymptomatic or may present with symptoms that depend on the location of the tumor. Among symptomatic patients, the most common symptoms are diminished vision and proptosis,...
Conclusion
Optic nerve glioma is a neoplasm that typically occurs in early childhood. However, a late-onset adult malignant form of optic nerve glioma exists and is a rare, highly aggressive visual pathway tumor that presents with bilateral visual loss and has a fatal outcome in virtually all cases. MRI is the imaging modality of choice.
Personal information and conflict of interest
A. Klemencic:
Nothing to disclose
M. Smoljan:
Nothing to disclose
D. Zadravec:
Nothing to disclose
References
1. Helmut Wilhelm. Primary optic nerve tumors. Curr Opin Neurol. 2009 Feb;22(1):11-8. PMID: 19155759
2. J Shapey, H V Danesh-Meyer, A H Kaye. Diagnosis and management of optic nerve glioma. J Clin Neurosci. 2011 Dec;18(12):1585-91. PMID: 22071462
3. Binning M J, Liu J K, Kestle J R W, Brockmeyer D L, Walker M L. Optic pathway gliomas: a review. Neurosurg Focus. 2007;23(5):E2. PMID: 18004964
4. Shofty B, Constantini S, Bokstein F, Ram Z, Ben-Sira L, Freedman S, Vainer G, Kesler A. Optic pathway gliomas in...