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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 22:1963-1969 (November 2001)
© 2001 American Society of Neuroradiology

BRAIN

Optic Pathway Glioma: Correlation of Imaging Findings with the Presence of Neurofibromatosis

Liora Kornreich^a, Susan Blaser^a, Michael Schwarz^a, Avinoam Shuper^a, Tal H. Vishne^a, Ian J. Cohen^a, Ricardo Faingold^a, Shalom Michovitz^a, Benjamin Koplewitz^a and Gadi Horev^a

^a From the Department of Imaging (L.K., M.S., G.H.) and the Center for Hematology-Oncology (A.S., I.J.C.), Schneider Children's Medical Center of Israel, Petah Tiqva, Israel; the Department of Imaging, Hospital for Sick Children, Toronto, Canada (S.B., R.F.); the Departments of Surgery (T.H.V.) and Neurosurgery (S.M.), Rabin Medical Center, Petah Tiqva, Israel; and the Department of Imaging, Hadassa Hospital, Jerusalem, Israel (B.K.).

BACKGROUND AND PURPOSE: Despite the benign histology of optic pathway glioma (OPG) (low-grade astrocytoma), its biological behavior is unpredictable, and it is unclear whether specific morphologic or anatomic patterns may be predictive of prognosis. It is also unclear whether OPG associated with neurofibromatosis (NF) is a distinct entity from non–NF-OPG. Our purpose was to describe the MR imaging features of OPG, compare the findings between patients with and those without NF, and identify prognostic imaging signs.

METHODS: MR examinations of 91 patients with OPG (47 with NF and 44 without) were reviewed at presentation and during follow-up. The images were evaluated for size and extension of tumor, and imaging parameters. Statistical bivariate analysis was used to compare the patients with and those without NF, and Pearson correlation was used to evaluate the correlation between the different imaging parameters and prognosis. Kappa values were calculated to determine intraobserver and interobserver variability.

RESULTS: The most common site of involvement in the NF group was the orbital nerve (66%), followed by the chiasm (62%). In the non-NF group, the chiasm was the most common site of involvement (91%); the orbital nerves were involved in only 32%. Extension beyond the optic pathway at diagnosis was uncommon in the NF group (2%) but frequent in the non-NF group (68%). In the NF group, the tumor was smaller and the original shape of the optic pathways was preserved (91% vs. 27% in the non-NF group). The presence of cystic components was significantly more common in the non-NF patients (66% vs. 9% in the NF group). During follow-up, half the NF patients remained stable, in contrast to 5% of the non-NF group. No statistical correlation was found between imaging features and biological behavior of the tumor.

CONCLUSION: NF-OPG is a separate entity from non–NF-OPG, with different imaging features and prognosis, thereby warranting a specific diagnostic, clinical, and therapeutic approach.

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