Juvenile ossifying fibroma (OF) is a benign fibro-osseous tumor, affecting the bones of the face. It’s locally very aggressive, with a strong tendency to recur. We report a case of a juvenile psammomatoid ossifying fibroma (JPOF) complicated with exophthalmos undergoing an entirely endoscopic resection.
A 14-years-old, young woman presented with a 7 months history of headache and right chronic tearing complicated with exophthalmos. Nasal endoscopy revealed a polylobed mass filling the right nasal cavity, the ophthalmic examination showed an isolated exophthalmos. CT scan revealed a well-limited benign mass covered by a thick shell of bone, pushing out the orbital lamina papyracea responsible for a grade 1 exophthalmos. On the facial MRI, we excluded intracranial or intraorbital involvement. A biopsy of the mass describes a psammomatoid juvenile ossifying fibroma. The patient underwent endoscopic transnasal approach with image-guided neuro-navigation system.
JPOF is an aggressive variant of ossifying fibroma occurring predominantly in children with a predilection for the paranasal sinuses. CT scan images show a characteristic well-limited benign expansile mass covered by a thick shell of bone, but sometimes it’s mistaken for a mucocele. MRI helps with excluding intracranial or intraorbital involvement. Endonasal endoscopic approaches have been increasingly used and it tends to become the new standard of care.
Treatment consists of complete surgical removal; incomplete resection is associated with a high local recurrence rate. Clinician should keep in mind the need for clinical and radiological follow-up for many years.

Copyright © 2021. Published by Elsevier Ltd.

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