CASE REPORT

Juvenile Ossifying Fibroma

Jithender Reddy Kubbi1, Navadeepak Kumar K1, Vivekanandh Reddy1, Ramlal G1

1 Department of Oral Medicine and Radiology SVS Institute of Dental Sciences Mahabubnagar, A.P ., India

ABSTRACT
Within the confusing ray of fibro-osseous lesions, the so called juvenile ossifying Fibroma (JAOF) is perhaps the most enigmatic. Pathologists considering such a diagnosis are faced with a plethora of subjective and arbitrary criteria and will find little consolidation in the literature. Occupying a subset within the spectrum of ossifying Fibroma. JAOF is considered by many to be a unique lesion because of its reported tendency to occur in children and adolescents, it's more complex histological features, and its purported tendency for locally aggressive growth. Because of this lesion's aggressive nature and high recurrence rate, early detection and complete surgical excision are essential. Reported here is a case of a massive juvenile ossifying fibroma of the maxilla in a 12year-old male child. Keywords: fibro-osseous lesions, juvenile ossifying Fibroma

Journal of Dental Sciences and Research Vol. 2, Issue 2, Pages 1-5

INTRODUCTION Juvenile ossifying fibroma (JOF) is a fibro-osseous neoplasm described as an actively growing lesion consisting of a cell-rich fibrous stroma, containing bands of cellular osteoid without osteoblastic lining, together with trabeculae of more typical woven bone. Small foci of giant cells may also be present. The lesion is nonencapsulated but well demarcated from surrounding bone (WHO)[1]. Although JOF was described in the past as a variant of ossifying Fibroma, but more recently it is segregated in two distinct sub types. 1) Trabecular - WHO type 2) psammomatoid variety, with varied clinical and histological features[2] the maxilla, paranasal sinus, orbit and fronto- ethmoid bone are the predilicted sites for the involvement of the JOF. Very few cases of mandibular JOF have been reported[3]. Clinically in most instances JOF is slowly growing asymptomatic swelling causing facial asymmetry, but tumor can progress to considerable size and behaves, as aggressive lesion. Less commonly, pain and parasthesia
Address for correspondence: Dr Jithender Reddy Kubbi E-mail: reddy_jittu@yahoo.co.in Access this article online Website: http://www.ssdctumkur.org/jdsr.php.

are noted. Depending upon anatomical site involvement, nasal obstruction, epistaxis and exopthalamus are observed. Slight male predominance with age ranging from 2- 15 years has been reported. The appearance of JOF radiographically varies from unilocular to multilocular radiolucency with well defined borders and occasionally opacification depending upon the stage and time of radiographic examination. The presence of cortical thinning, perforation, tooth displacement and root resorption, are suggestive of feature of aggressive JOF. The advanced imaging modalities like CT and MRI have show to reveal more invasive and destructive features of JOF apart from conventional radiographic features[4]. Histopathalogically both sub- types are typically nonencapsulated but well demarcated from surrounding bone. The tumor consists of varying neoplastic cellular stroma formed by spindal or oval shaped fibroblast cells. The mineralized component in the both patterns is distinct. The trabecular variant shows irregular strands of highly cellular osteoid encasing plump and irregular osteocytes. The plump osteoblasts often line the strands and focal areas of multinucleated giant cell are also observed. In contrast psammomatoid pattern forms concentric laminated and spherical ossicles that vary in shape and typically have basophilic centers with peripheral eosinophilic osteoid rims[5].

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The overall clinical, imaging and histopathological features are required to label the diagnosis of JOF. Because of unpredictable rapid and progressive growth of some JOF, the management and the prognosis of JOF are uncertain. Non- aggressive JOF are treated by curettage and local excision. Aggressive JOF require the complete surgical excision, en-bloc or hemimaxillectomy to prevent recurrences.6 Reported here is a case of JOF WHO type of maxilla occurring in a 14 yrs old boy. CASE REPORT A 14 yrs old boy who reported to the department of oral medicine and radiology in S.V.S Institute of Dental sciences Mahabubnagar Andhrapradesh INDIA. Had a chief complaint of painless, slowly progressive swelling of left side of face with 3 yrs duration. Initially to begin with swelling was of areca nut size and increased progressively to attain final size causing significant facial asymmetry without any associated symptoms. Other medical and dental histories were noncontributory. On extra oral examination a solitary, bony hard, painless, diffused swelling of approximately 4x4 cm on the left side of face. (Fig 1) Intraorally a solitary swelling causing obliteration of left buccal vestibule with overlying normal mucosa with palatal expansion noted. (Fig 2) Lateral occlusal radiograph revealed a well defined, homogenous, complete radiopaque lesion occupying left maxillary sinus with evidence of complete sinus opacification and expansion of cortices. (Fig.3) Computed tomography findings confirmed routine radiographic features and further showed evidence of erosion of maxillary bony wall and extension to cheek anterio-inferiorly.(Fig 4) Based upon clinical and radiographic examination provisional diagnosis of as JOF of left maxilla with differential diagnosis of fibrous dysplasia. Pre-operative incisional biopsy was performed and histopathological diagnosis of JOF- WHO type was given. (Fig 5) The lesion exposed and excision by partial maxillectomy with thoroughly curetted and closer was done using 3.0 vicryl sutures and acrylic splint was placed.
Figure 2: Intraorally showing expansion of cortices.

Figure 1: Extra oral photograph showing facial asymmetry on the left side of the Face.

Figure 3: Lateral occlusal radiograph showing expansile lesion on the left maxillary Sinus with Ossifications.
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Figure 4: CT scan reveals large tumour involving left maxilla and maxillary sinus With ossifications.

Figure 5: Trabeculae of cellular woven bone are present.

DISCUSSION JOF commonly affects young children with slight male predilection with a mean age of 11 years. The age of the patient in this case was 14 years which is in-concurrent with the previous reports. Clinically JOF is asymptomatic most of the time, however less commonly it is aggressive and symptomatic. This asymptomatic nature, was also appreciated in this present case.8Although both maxilla and mandible are affected with slight predominance for maxilla has been suggested by Pieter j.slootweg.9 In the present case swelling of duration of six months was noted. The variation in the duration was reported from slowly growing over a periods of years to rapid increase in weeks. The spectrum of radiographic features of JOF, varies from a well defined unilocular to multilocular with central opacification or ground glass appearance.2,9 This radiographic variation of JOF is due to stage, duration and histology of the neoplasm. The 50% of cases of sinus JOF are reported in multiple sinuses with remaining occurring in single sinus.10 The JOF also was seen in single sinus. The clinical findings, radiographic features, duration and extension of the lesion in to soft tissue favours the aggressive type of JOF as previously reported.3 The features like tooth displacement, resorption and perforation of cortices or bone in addition to rapid growth of the lesion favors the diagnosis of aggressive variety of JOF. However in this case except tooth resorption all other features like tooth displacement and perforation were noted. CONCLUSION Although juvenile ossifying Fibroma an uncommon clinical entity, its aggressive social behavior and high recurrence rate means that is important to make an early

diagnosis, apply the appropriate treatment and long term and follow up. REFERENCES
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