J Korean Ophthalmol Soc.  2014 Oct;55(10):1543-1548. 10.3341/jkos.2014.55.10.1543.

A Case of Huge Pilocytic Astrocytoma Causing Eyeball Subluxation

• Kim EY
• Jung SK
• Paik JS
• Yang SW

• KMID: 2216876
• DOI: http://doi.org/10.3341/jkos.2014.55.10.1543

Abstract

PURPOSE
To report a relatively rare case of huge pilocytic astrocytoma of the optic nerve and optic chiasm causing eyeball subluxation.
CASE SUMMARY
An eight-year-old male presented with proptosis and visual loss in the left eye for one year. The radiological findings showed a 2.9 x 2.7 x 4.2-cm tumor on the left optic nerve and optic chiasm. For diagnosis and treatment, the patient underwent tumor resection and enucleation. Pathohistological analysis of the tumor specimen revealed pilocytic astrocytoma, which is classified by the World Health Organization as a grade I astrocytic tumor.
CONCLUSIONS
Astrocytoma is a tumor of the brain that affects children more often than adults. In general, gross-total resection of pilocytic astrocytoma is expected to be curative due to the non-invasive feature of the tumor. Considering pilocytic astrocytoma as differential diagnosis of orbital tumor in children with symptoms of rapidly progressive proptosis and decreased visual acuity is important because occurrence in the optic nerve and optic chiasm is possible.

Keyword

Optic chiasm; Optic nerve; Pilocytic astrocytoma

MeSH Terms

Adult
Astrocytoma*
Brain
Child
Diagnosis
Diagnosis, Differential
Exophthalmos
Humans
Male
Optic Chiasm
Optic Nerve
Orbit
Visual Acuity
World Health Organization

Figure

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  Figure 1. (A) Photograph of the patient taken at the first visit to the hospital shows severe proptosis and subluxation of the left eye. (B) Left eyeball is infero-laterally deviated (40-PD LXT, LHT by Hirschberg test).

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  Figure 2. Photographs show limitation of the left eyeball movement for adduction and upward gaze. (A) Right side gaze, (B) left side gaze, (C) downward gaze, (D) upward gaze.

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  Figure 3. (A) 2.9 × 2.7 × 4.2-cm-sized enhancing mass involving the left optic nerve and optic chiasm (Orbit MRI (T1-weighted fat-suppressed gadolinium-enhanced image): A: axial view; B: coronal view; C: sagittal view).

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  Figure 4. Gross specimen of the optic nerve and optic chiasm astrocytoma, measuring 3.3 × 3.0 × 2.3 cm.

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  Figure 5. (A) Round and piloid cells constitute this tumor; Rosenthal fibers (arrow) is seen (H&E stain, ×100). (B) Immunohistochemical stains (×100), positive for (B-1) CD34, (B-2) Glial Fibrillary Acidic Protein (GFAP), (B-3) Synapto- physin, (B-4) Oligodendrocyte transcription factor (OLIG-2).

Reference

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