Psammomatoid Juvenile Ossifying Fibroma: A Case Report with Literature Review

Maxillofacial fibro-osseous lesions are a generic designation of a group of jaw disorders characterized by the replacement of bone by a connective tissue matrix, which displays varying degrees of mineralization in the form of woven bone or of cementum-like structures indistinguishable from “cementicles”.1 These lesions are of particular interest to the maxillofacial radiologist because they emphasize the role of radiologist in the diagnostic process. In general, fibro-osseous lesions of the maxillofacial region present a diagnostic dilemma for the clinician and pathologist owing to the overlapping clinical and histomorphologic findings. Juvenile ossifying fibroma, like ossifying fibroma, is a central lesion of bone composed of variable amount of fibrous tissue proliferation and foci of calcification.2 Grossly the juvenile ossifying fibroma appears as a discrete mass well demarcated from the surrounding bone. In the second edition of WHO classification of odontogenic tumors 1992, psammomatoid juvenile ossifying fibroma is classified under “Neoplasms and other lesions related to bone”.1 The prefix juvenile indicates its occurrence in younger individuals. The term active refers to clinical aggressive behavior, such as bone erosion and soft tissue invasion. The most common clinical manifestation is proptosis. Other symptoms include nasal obstruction, headache, swelling and rarely epistaxis. Two histological patterns are classically described: (1) trabecular and (2) psammomatoid.2-4 Psammomatoid juvenile ossifying fibroma is an aggressive variant of ossifying fibroma usually seen in the pediatric age group that is histologically characterized by the formation of psammoma body islands.2 The word “psammos’’ is derived from the Greek word meaning “sand”.5 The most distinctive component in these lesions is the presence of calcified ossicles containing osteocytes known as psammomatoid bodies. Psammomatoid juvenile ossifying fibroma (PJOF) has its peak incidence in the first and second decades of life with a slight male predilection. The preferred sites are paranasal sinuses, maxilla, ethmoid and frontal bones.3