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7-Year-Old Female with Left Eye Proptosis
This case features a 7-year-old female presenting with left eye proptosis, ultimately diagnosed with juvenile cemento-ossifying fibroma (JCOF) of the ethmoid sinus.
Imaging overview:
Axial T2, axial T1 post-contrast MRI, and axial contrast-enhanced CT of the orbits and paranasal sinuses demonstrate a well-demarcated mass centered in the ethmoid sinus with classic egg-shell calcification and variable internal density on CT. MRI shows a peripherally enhancing mass with T1 intermediate and T2 hypointense signals reflecting the cementum and osteoid matrix, without restricted diffusion on DWI.
Clinical insight:
Juvenile cemento-ossifying fibroma is a rare variant of ossifying fibroma containing both cementum and osteoid matrix, most commonly arising in the paranasal sinuses of children and adolescents. Unlike conventional ossifying fibroma, JCOF tends to behave in a locally aggressive fashion with a high recurrence rate following resection. On CT, the characteristic egg-shell calcification pattern and well-demarcated margins help distinguish it from other fibro-osseous lesions, while MRI provides complementary information about soft tissue extent and orbital involvement. Early recognition is important given its aggressive local behavior and surgical implications.
Case courtesy of Abel T. Abebe, MD and Avin Camacho, MD,
The University of Texas Medical Branch at Galveston.