Intracranial extended Psammomatoid Juvenile Ossifying Fibroma: case report and systematic review

Abstract

Psammomatoid Juvenile Ossifying Fibroma is an uncommon fibro-osseous neoplasm of aggressive but benign nature found in the younger age. Its aggressive path can lead to facial deformation, eye proptosis, and development of intracranial extensions leading to various neurological symptoms. A systematic review based by the MOOSE guideline in Medline, EMBASE and Lilacs, resulted in 23 reported cases of intracranial extended PJOF. Hence, we found it relevant to present a case report of a 15-year-old male with facial deformation and left eye proptosis absent of visual disturbances with PJOF. The lesion was present in the left anterior base of the skull and extended to the intra-orbital space and over the zygomatic arch. The diagnosis was only confirmed as PJOF by histopathological analysis of the completely resected lesion. Follow up visits documented unremarkable regression of the facial deformity and post-op images showed a completely resected lesion. Our case raises the need to be aware of this rare tumor that can be confused with a meningioma or other intracranial tumors.