Acute lymphoblastic leukemia with optic pathway involvement

Case contributed by Ammar Haouimi


Patient under chemotherapy for acute lymphoblastic leukemia (ALL), presented with a two-month history of decreased visual acuity and left proptosis

Patient Data

Age: 5 years
Gender: Male

Left proptosis (axial grade I) with retinal hemorrhage of high signal intensity on T1WI and FLAIR, and low signal intensity on T2WI and GE with no significant enhancement seen on post-contrast sequences. Diffuse thickening of the left optic nerve with perineural enhancement and intraconal fat infiltration. 

The right orbit as well as the optic chiasma appear normal.

Patient still under oral chemotherapy. Follow-up MRI performed 9 months later shows diffuse thickening of both optic nerves more pronounced on the left with perineural enhancement and intraconal fat infiltration. There is a lobulated opto-chiasmatic mass isointense to the gray matter on both T1WI/T2WI with relatively homogeneous enhancement on postcontrast sequences, compressing the 3rd ventricle with mild dilatation of the lateral ventricles with periventricular interstitial edema (obstructive hydrocephalus).

Reduced volume of the left globe, with progression of the vitreous hemorrhage extending to the anterior segment with internal septation.

Case Discussion

The 1st MRI shows features of retinal hemorrhage with leukemic infiltration of the left optic nerve in a patient with an acute lymphoblastic leukemia (ALL).

The 2nd MRI (performed 9 months later) shows progression of the orbital involvement with extension to the contralateral orbit, and appearance of a tumoral process at the opto-chiasmatic region highly suggestive of bilateral optic nerves/opto-chiasmatic gliomas.

Orbital involvement is the result of an infiltration of soft tissue or orbital bone, usually seen up to 75%  of acute lymphoblastic leukemia (ALL), and up to 20% of acute myeloid leukemia (AML). MRI is more sensitive than CT in the detection of blood and blood products within the globe.

Tumoral processes such as meningiomaglioma and sarcoma are usually seen after radiation-therapy (radiation-induced neoplasms), however, they are also described in patients who have not received radiation-therapy 1 (as in this case).


Additional contributor: R. Bouguelaa, MD

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