fibro - osseous lesions of the jaws represent a diverse group of entities which include developmental ( hamartomatous ) lesions , reactive or dysplastic processes and neoplasms . benign fibro - osseous lesions of the head and neck region are uncommon and represent a wide range of tumors sharing some histopathological features ; which comprise fibrous dysplasia , ossifying fibroma ( of ) , and cement - osseous dysplasia . of can be further divided into conventional and juvenile forms ( juvenile ossifying fibroma ( jof ) ) . jof has to be distinguished from a larger group of ofs on the basis of the age of the patient , site of involvement and clinical behaviour . according to the world health organization ( who ) classification of odontogenic tumors 2005 , jof is further subdivided into psammomatoid jof ( psjof ) and trabecular jof ( trjof ) . benjamins in 1938 first reported psjof as an  osteoid fibroma with atypical ossification of the frontal sinus , golgi in 1949 called it as  psammomatoid ossifying fibroma  , and johnson in 1952 coined the term  juvenile active ossifying fibroma  . according to who classification of odontogenic tumors 2005 , it was named as the term juvenile psammomatoid ossifying fibroma is now used to designate the neoplasm of the craniofacial skeleton of young age with well - defined clinicopathological features . the pathogenesis of these jaw lesions are related to the abnormal development of basal generative mechanism that is essential for root formation . this paper highlights a rare entity of psjof involving the maxillary sinus and nasal cavity on the left side . a 20-year - old female patient reported with a painless progressive swelling in the left cheek region and difficulty in breathing since 1 year . past medical history revealed that the patient underwent surgery in a private dental clinic 5 years back for the impacted tooth 23 which was associated with pathology . the swelling was 6  4 cm in size approximately extending anteroposteriorly from ala of the nose to 5 cm from tragus of the ear on the left side . nasal polyp was seen in the left nostril with obliteration of left ala of the nose [ figure 1 ] . nasal polyp seen in left nostril with obliteration of left ala of nose intraoral examination presented a swelling extending anteroposteriorly from the distal aspect of 21 to mesial aspect of 26 ; medially till the mid palatine raphe and laterally the buccal vestibule was obliterated and the swelling extended from 21 to 26 . paranasal sinus ( pns ) x - ray revealed haziness in the left maxillary sinus . computed tomography ( ct ) scan confirmed well - defined mixed radiolucent and radiopaque areas with calcifications extending superoinferiorly from infraorbital rim to alveolus and anteroposteriorly from the nasal septum to post zygomatic buttress on left side [ figure 2 ] . axial computed tomography revealing well - defined radiolucent and radiopaque areas with calcifications histopathological examination of incisional biopsy revealed connective tissue stroma with numerous spherical / irregular ossifications interspersed with cellular fibrous tissue [ figure 3 ] . the ossifications showed peripheral brush border surrounded by an eosinophilic rimming [ figure 4 ] . haemorrhagic areas were also seen . the constellation of clinical , radiological , and histopathological features of this lesion supported an interpretation of psammomatoid variant of jof . photomicrograph of hematoxylin and eosin ( h&e ) stained section ( 100 ) showing numerous spherical and irregular calcifications interspersed with fibrous tissue photomicrograph of h&e stained section ( 400 ) showing peripheral brush border surrounded by eosinophilic rimming under general anesthesia , a weber - ferguson incision was given to expose the complete lesion . subtotal maxillectomy was performed and the tumor mass along with nasal and ethmoidal polyps were removed with the help of a chisel and mallet . after complete removal of the mass , borders were carefully osteotomized to avoid the chances of recurrence [ figure 5 ] . the excised specimen was sent for histopathological examination [ figure 6 ] and the diagnosis of psjof was confirmed . jof are benign , potentially aggressive fibro - osseous lesions of the craniofacial bones . the word psjof is a unique variant of jof that has a predilection for orbit and pnss accounting for about 72% followed by calvarium 11% , maxilla 10% , and mandible 7% . the ethmoidal sinuses are most commonly involved , followed by the frontal sinuses , the maxillary sinuses , and the sphenoid sinus . both the variants of jof show a predilection for males . the swelling in this case is associated with maxilla involving maxillary sinus and nasal cavity in a 20-year - old female patient . table 1 summarizes the clinical presentation of case series of psjof reviewed in the literature . the patients may develop exopthalmoses , bulbar displacement , and nasal obstruction . in the present case because of the extension of the tumor into the nasal cavity , nasal obstruction was present on the left side . radiographically , psjof can be radiolucent , radiopaque , or mixed depending upon cystic changes and the degree of calcification . sclerotic changes are evident in the lesion which may show a ground - glass appearance . histologically , psjof shows highly cellular fibrous stroma often with whorled pattern containing closely packed spherical ossicles resembling psammoma bodies . these ossicles are round to oval in shape which have a basophilic center and peripheral pink rim showing some radiating fibers which corroborates with the microscopic features provided in the present case . psjof should be differentiated from extracranial meningioma with psammoma bodies , which demonstrates epithelial membrane antigen ( ema ) positivity and even the psammomatoid ossicles in psjof are clearly different from spherical true psammoma bodies . other differential diagnosis include fibrous dysplasia , osteoma , cementoblastoma , well - differentiated osteosarcoma , psammomatous extracranial meningioma [ table 2 ] . differential diagnosis the clinical management of smaller lesions is simple excision with surrounding marginal bone , whereas larger lesions warrant more aggressive surgical management . prognosis is good with a recurrence rate of about 30 - 58% . no malignant transformation has been documented . even though these lesions tend to locally invade , there were no cases of metastasis being reported . therefore , it is very important to correlate the clinical , radiographical , and histopathological findings for proper treatment . early detection and complete surgical excision of the lesion followed by long - term follow - up is necessary for proper clinical management .
juvenile ossifying fibroma ( jof ) is an uncommon , benign , bone - forming neoplasm with an aggressive local growth that is distinguished from other fibro - osseous lesions primarily by its age of onset , clinical presentation and aggressive behaviour . 
 jof is considered as a variant of the ossifying fibroma ( of ) and the former includes psammomatoid jof ( psjof ) and trabecular jof ( trjof ) . 
 both variants involve the craniofacial bones with the trabecular variant being more common in the jaws and the psammomatoid variant being more common in the craniofacial skeleton . 
 psjof is an unique variant of jof that has a predilection for the sinonasal tract and the orbit particularly centered on the periorbital , frontal , and ethmoid bones . 
 we report a rare case of massive psjof involving the maxillary sinus in a 20-year - old female .