Optic Pathway Glioma MRI

Optic Pathway Glioma MRI

Usually showing low T1 and high central T2 signal on MRI images, enhancement is variable.

MR imaging is optimal for showing the relationship of the mass to the hypothalamusoptic chiasm, and infundibulum as well as the intraorbital and intracanalicular components of the mass. Large tumours are typically heterogeneous with cystic and solid components.

T1: enlargement, often iso to hypointense compared to the contralateral side

T2: hyperintense centrally

thin low-signal at the periphery representing the dura 1).

FLAIR: hyper intense

T1 C+ (Gd): enhancement is variable

Patients with chiasmatic-hypothalamic low-grade glioma (CHLGG) have frequent MRIs with gadolinium-based contrast agents (GBCA) for disease monitoring. Cumulative gadolinium deposition in the brains of children is a potential concern. The purpose of a study of Malbari et al. was to evaluate whether MRI with GBCA is necessary for determining radiographic tumor progression in children with CHLGG.

Children who were treated for progressive CHLGG from 2005 to 2019 at Texas Children’s Cancer Center were identified. Pre- and post-contrast MRI sequences were separately reviewed by one neuroradiologist who was blinded to the clinical course. Three-dimensional measurements and tumor characteristics were evaluated. Radiographic progression was defined as a 25% increase in size (product of two largest dimensions) compared with baseline or best response after initiation of therapy.

A total of 28 patients with progressive CHLGG were identified with a total of 683 MRIs with GBCA reviewed (mean 24 MRIs/patient; range, 11-43 MRIs). Radiographic progression was observed 92 times, 91 (99%) on noncontrast and 90 (98%) on contrast imaging. Sixty-seven progressions necessitating management changes were identified in all (100%) noncontrast sequences and 66 (99%) contrast sequences. Tumor growth > 2 mm in any dimension was identified in 184/187 (98%) noncontrast and 181/187 (97%) with contrast imaging. Metastatic tumors were better visualized on contrast imaging in 4/7 (57%).

MRI without GBCA effectively identifies patients with progressive disease. When imaging children with CHLGG, eliminating GBCA should be considered unless monitoring patients with metastatic disease 2)


Gadolinium might not be needed for these exams to inform management decisions. Secondary benefits of a non-contrast follow-up protocol include decreased cost and risk to the patient 3).


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 4).


1)

Müller-Forell WS, Boltshauser E. Imaging of Orbital and Visual Pathway Pathology. Springer Verlag. (2005) ISBN:3540279881.
2)

Malbari F, Chintagumpala MM, Wood AC, Levy AS, Su JM, Okcu MF, Lin FY, Lindsay H, Rednam SP, Baxter PA, Paulino AC, Orzaiz GA, Whitehead WE, Dauser R, Supakul N, Kralik SF. Gadolinium is not necessary for surveillance MR imaging in children with chiasmatic-hypothalamic low-grade glioma. Pediatr Blood Cancer. 2021 Jun 16:e29178. doi: 10.1002/pbc.29178. Epub ahead of print. PMID: 34133064.
3)

Maloney E, Stanescu AL, Perez FA, Iyer RS, Otto RK, Leary S, Steuten L, Phipps AI, Shaw DWW. Surveillance magnetic resonance imaging for isolated optic pathway gliomas: is gadolinium necessary? Pediatr Radiol. 2018 Sep;48(10):1472-1484. doi: 10.1007/s00247-018-4154-4. Epub 2018 May 22. PMID: 29789890.
4)

Kornreich L, Blaser S, Schwarz M, Shuper A, Vishne TH, Cohen IJ, Faingold R, Michovitz S, Koplewitz B, Horev G. Optic pathway glioma: correlation of imaging findings with the presence of neurofibromatosis. AJNR Am J Neuroradiol. 2001 Nov-Dec;22(10):1963-9. PMID: 11733333; PMCID: PMC7973845.

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