Juvenile Psammomatous Ossifying Fibroma

An 18-year-old female presented with blurry vision, headaches for 4 months. She was diagnosed to have papilledema on an outside eye exam and referred to BPEI. Her visual acuity was 20/20 OD, 20/100 OS. Additionally, she was noted to have junctional scotoma on visual fields and papilledema on fundus evaluation. Her OCT RNFL showed thickening both eyes, OCT GCC showed diffuse loss in left eye and superotemporal loss in right eye. These findings prompted neuroimaging. Her MRI brain showed anterior skull base tumor that pushed pituitary posteriorly. She underwent endoscopic trans-sphenoidal resection of the tumor. Histopathology of the tumor showed juvenile ossifying fibroma, psammomatoid variant. Although JOF is previously described in the craniofacial region, there is only one case report of sellar mass due to JOF in a 15-year-old and another with orbital invasion in two cases. At a subsequent visit she had improvement in her visual acuity and a decrease in her papilledema. We describe an unusual case of JOF as an anterior skull base mass causing junctional scotoma and papilledema in an 18-year-old and our experience with management of this rare tumor.

Presentation Date: 08/27/2020

Issue Date: 02/05/2021