Juvenile psammomatoid ossifying fibroma of OrbitA rare case report and review of literature

Abstract

Fibro osseous lesions of the craniofacial skeleton are a benign condition in which the normal architecture of the bone is replaced by fibrous connective tissue with varying degrees of mineralization. JOF forms a special entity among the fibro osseous lesions because of its age of occurrence and its aggressive nature thereby mimicking a malignancy. The Juvenile Ossifying Fibromas were further subdivided into Psammomatoid and Trabecular variant based on their histopathological characteristics. They tend to differ in their mineralized portion with the trabecular variant showing woven bone while the psammamotoid shows lamellated and spherical ossicles in various shapes in a myxoid stroma intermingled with bone cyst like areas. The reported cases of JPSOF are few, hence histopathological examination is a valuable tool in the diagnosis of this rare lesion and JOF should always be considered in the differential diagnosis of the lesions of the craniofacial skeleton. Early diagnosis and a complete surgical excision with adequate margins and a long term follow up is mandatory for a good prognosis of this highly recurrent and aggressive lesion.The diagnosis of JOF requires a careful correlation of clinical, radiological and histopathological features. The purpose of this paper is to report a case of JPOF of the orbit to stress the need for consideration of JOF in the differential diagnosis of the aggressive lesions of the Cranio facial skeleton.