Department of Pathology, University of Oklahoma Health Sciences Center

 

December 2004, Case 412-2. Quiz set! Click here to see.

A 12 year-old girl with an enlarged optic nerve.

Clinical information The patient was a 12 year-old girl who started to experience slowly progressive visual loss in the left eye 18 months ago. MRI demonstrated enlargement of the optic nerve by a fusiform  tumor. The tumor is slightly hypointense on T1-weighed images and slightly hyperintense on T2-weighed images. There was no extension of the tumor beyond the orbit. The hypothalamus and optic chiasma were within normal limits. The left globe and the tumor was completely enucleated. At the time of surgery, the left eye was completely blind.

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What is your diagnosis? This condition is most often associated with what syndrome? Discussion

Cases of the Month  Evaluation  Coordinator: KarMing-Fung@ouhsc.edu