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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 26  |  Issue : 2  |  Page : 213-218

A case report on juvenile ossifying fibroma of the mandible


Department of Oral Medicine and Radiology, Government Dental College and Research Institute, Bangalore, Karnataka, India

Date of Submission13-Jun-2014
Date of Acceptance27-Sep-2014
Date of Web Publication30-Oct-2014

Correspondence Address:
Preeti Rajguru
Department of Oral Medicine and Radiology, Government Dental College and Research Institute, Bangalore - 560 002, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.143706

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   Abstract 

Juvenile ossifying fibroma is considered a unique entity occurring primarily in children and adolescents. Although a benign neoplasm of bone origin, it is an aggressive variant of ossifying fibroma of the jaws. On account of the variable clinical behavior, a highly aggressive nature, and a high tendency for recurrence, early detection and prompt treatment is required. This report presents a case of juvenile ossifying fibroma in an eight-year-old girl, who reported to our institution with a gradually progressive swelling on the left side of her face since nine months, with typical clinical, radiological, and histopathological features.

Keywords: Fibro-osseous lesion, juvenile ossifying fibroma, ossifying fibroma


How to cite this article:
Khan M, Ramachandra VK, Rajguru P. A case report on juvenile ossifying fibroma of the mandible . J Indian Acad Oral Med Radiol 2014;26:213-8

How to cite this URL:
Khan M, Ramachandra VK, Rajguru P. A case report on juvenile ossifying fibroma of the mandible . J Indian Acad Oral Med Radiol [serial online] 2014 [cited 2019 Mar 24];26:213-8. Available from: http://www.jiaomr.in/text.asp?2014/26/2/213/143706


   Introduction Top


Juvenile ossifying fibroma (JOF) is a rare fibro-osseous entity of the craniofacial skeleton, which poses diagnostic and therapeutic challenges, due to its characteristic behavioral, clinical, and histopathological features. The second edition of the World Health Organization (WHO) Classification of odontogenic tumors defines JOF as a lesion consisting of cell-rich fibrous tissue containing bands of cellular osteoid without osteoblastic rimming with trabeculae of more typical woven bone. [1]

The juvenile ossifying fibroma was first described by Sir Benjamin, in 1938, as an 'osteoid fibroma with atypical calcification' and later on in 1952, Sir Johnson et al., for the first time, coined the term 'juvenile active ossifying fibroma'. [2] In literature it is known by a variety of terms: Juvenile active ossifying fibroma, juvenile aggressive ossifying fibroma, trabecular osteo desmoblastoma, and active fibrous dysplasia.

Juvenile ossifying fibroma is a benign bone forming neoplasm, which is defined as a variant of ossifying fibroma occurring in the craniofacial skeleton of young patients. [3] It arises within the craniofacial bones of children under the age of 15 years; however, there are reports of ossifying fibromas in patients with ages ranging from three months to 72 years. [4] JOF differs from the adult variant of ossifying fibroma on the basis of the age of occurrence, anatomic site of involvement, high tendency for recurrence, and its locally aggressive behavior.


   Case Report Top


An eight-year-old girl reported to our department with a swelling in the lower left side of her face since nine months. The swelling was initially small in size and had gradually increased to attain the present size, without any associated symptoms. The dental history revealed that the patient had visited a private hospital for the same, where a biopsy was carried out, the reports of which were not available. There was no history of trauma or any other surgical intervention in the past. The patient's medical and family histories were noncontributory. A general physical examination revealed that the patient was undernourished and poorly built.

An extraoral examination revealed gross facial asymmetry caused by a diffuse, smooth-surfaced, non-erythematous swelling, measuring approximately 3 × 3 cm in dimension, involving the left parasymphysis, and extending to the body of the mandible [Figure 1]a and b. The swelling was uniformly firm in consistency, nontender, without crepitus, and not adherent to the overlying skin. There was no paresthesia or lymphadenopathy associated with the swelling.

The intraoral examination revealed adequate mouth opening with a mixed dentition status and vestibular obliteration with respect to the left lower lateral incisor to the first molar, owing to expansion of the buccal and lingual cortical plates [Figure 2]. The swelling was uniformly firm and nontender, without crepitus on palpation. The overlying mucosa was normal without secondary changes. There was no evidence of draining fistulae or sinuses, or mobility or displacement of the teeth in this region.
Figure 1: (a) Lateral view of the extraoral swelling in the left mandibular region. (b) Inferior view of the extraoral swelling in the left mandibular region

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Figure 2: Intraoral view showing obliteration of the lower left buccal vestibule from the 32 to 36 region

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On the basis of the history and clinical examination we arrived at a provisional diagnosis of juvenile ossifying fibroma of the left side of the mandible. Differential diagnoses of cemento-ossifying fibroma, osteoblastoma, and fibrous dysplasia were considered.

Radiographic evaluation

Intraoral periapical radiographs (IOPAR) revealed a large, ill-defined, non-uniform, mixed radiopaque-radiolucent lesion, extending from the 74 to 37 region, with a displaced tooth bud in relation to 35. The mandibular occlusal radiograph revealed expansion of both the buccal and lingual cortical plates on the left side of the mandible [Figure 3]. An orthopantomograph revealed a large, single, well-defined, unilocular expansile lesion, with a sclerotic border, containing multiple flecks of calcification in the left mandible extending from 32 to the tooth bud of 37, measuring about 3 × 3 cm in its greatest dimension. There was ballooning and thinning of the inferior cortex of the mandible. There was displacement of 34 and root resorption with respect to 74, 75, and 36. The tooth bud of 35 was displaced [Figure 4].
Figure 3: Mandibular cross-sectional occlusal view showing expansion of both the buccal and lingual cortical plates

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Figure 4: Orthopantomograph showing mixed radiolucent– radiopaque lesion in the left mandibular premolar– molar region, with flecks of calcification, displaced 35 tooth bud, with downward bowing and thining of the inferior border of the mandible

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Cone-Beam Computed Tomography (CBCT) revealed a radiolucent area lined by thin sclerotic margins in most of the areas, roughly measuring about 3 cm anteroposteriorly, 2.5 cm labiolingually, and 2.5 cm superoinferiorly, in the left mandibular body extending from the 31 region posteriorly. The radiolucency was not uniform within; there were diffuse specks of radiopaque areas within the lesion. The radiodensity was similar to that of the bone and also showed a trabecular pattern with severe expansion of the labial and lingual bone, thus causing severe thinning and displacement of the inferior border of the mandible inferiorly [Figure 5]. It also displaced the mandibular canal more labially [Figure 6]. The 3D reconstructed CBCT image showed ballooning of the inferior border of the mandible with a buccally placed 34 [Figure 7]. Radiographic diagnosis of a juvenile ossifying fibroma and radiographic differential diagnosis of cemento-ossifying fibroma, osteoblastoma, and cementoblastoma was established. The routine blood investigations were within normal limits and a posteroanterior chest radiograph was made to rule out any distant metastasis.
Figure 5: Axial section CBCT image showing anteroposterior and labiolingual extension of the lesion

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Figure 6: Coronal section CBCT image showing displacement of the inferior alveolar nerve canal buccally

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Figure 7: 3D reconstructed image showing ballooning of the inferior border of the mandible with buccally placed 34

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An incisional biopsy was subsequently performed providing a diagnosis of trabecular juvenile ossifying fibroma, showing cell-rich fibrous stroma containing strands of a cellular osteoid without osteoblastic rimming along with few trabeculae of a typical woven bone [Figure 8]. Hence, a final diagnosis of trabecular juvenile ossifying fibroma was established. The patient was managed surgically by mandibular resection with reconstruction, under general anesthesia, as the mass was extensive [Figure 9]. A long-term follow-up was planned, because of its known high recurrence rate. The patient was reviewed regularly and on a follow-up of one year, the lesion showed no signs of recurrence [Figure 10].
Figure 8: Hematoxylin and eosin stained section showing cell-rich fibrous stroma containing strands of cellular osteoid without osteoblastic rimming with few trabeculae of a typical woven bone

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Figure 9: Excised surgical specimen

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Figure 10: Postoperative orthopantomograph of the patient

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   Discussion Top


Ossifying fibroma (OF), a rare tumor, is a well-demarcated benign fibro-osseous tumor with a capsule composed of metaplastic bone, fibrous tissue, and a varying amount of osteoid tissue. Ossifying fibromas are subdivided into conventional and juvenile subtypes. [5] The JOF is an uncommon fibro-osseous neoplasm, is seen considerably less frequently, and behaves in a more aggressive fashion than its conventional counterpart.

The etiology of an OF is unknown, but odontogenic, developmental, and traumatic origins have been suggested, and it is also thought to be of a periodontal ligament origin because of its capacity to produce cementum and osteoid material. [6] Association of a new tumor suppressor gene (HPRT2) has been reported by Piementa et al., suggesting that the lesion could arise as a result of haploinsufficiency of the particular gene. [1] It has been hypothesized that a juvenile psamommatoid ossifying fibroma (JPsOF) originates from overproduction of the myxofibrous cellular stroma. These stromal cells secrete hyaline material that ossifies the connective tissue mucin that initiates the cystic areas. [6]

Gender predilection has been a matter of controversy, with some authors claiming no predilection for either sex, whereas, Johnson et al. found a higher incidence in females and El-Mofty reported a male predilection. [7]

The clinical presentation of JOF is a slow growing, asymptomatic, expansile, spherical or ovoid swelling, causing significant facial asymmetry, and depending upon the anatomical site of involvement there can be nasal obstruction, epistaxis, exopthalamus, and proptosis. In most patients (85%), the tumors are located in the facial bones, but they also involve the calvaria (12%) and extracranial sites (4%). In the facial bones, 90% of the tumors arise from the maxilla and paranasal sinuses and the remaining 10% arise from the mandible. [8] Rarely pain and paresthesia associated with the swelling are present. Root resorption and displacement of the involved teeth are observed. In the mandible, the angle and ramus are the most common sites of involvement, [3] which is in contrast to the reported case, wherein the parasymphysis and body of the mandible were affected.

Radiographic features are highly variable depending upon the stage of development and amount of calcification. Varying degrees of radiolucency and opacity are commonly seen, along with a fair demarcation of the tumor. [9] Radiographically, it is characterized by three stages. Stage I or the initial stage appears as a well-defined radiolucency, with no evidence of internal calcification. Stage II or mixed stage is characterized by flecks of radiopacities in the radiolucent area. Stage III or the mature stage is a completely radiopaque mass. Erosion and invasion of the surrounding bone may be noted in more aggressive cases. [9] Our case also documents the classical radiographic features of this rare entity. Radiographically, our case corresponds to stage II, that is, the mixed stage showing flecks of radiopacities.

On computed tomographic (CT) scans, juvenile ossifying fibromas appear as an expansile mass, surrounded by thick or thin radiodense rimming. There may be islands of bone formation within the lesion. On magnetic resonance imaging (MRI) scans, juvenile ossifying fibromas appear heterogeneous and usually have low-to-intermediate signal intensity on T1-weighted images and hypointense signal intensity on T2-weighted MR images. There is moderate contrast enhancement on postgadolinium T1-weighted MRI scans. Large mandibular lesions may cause a characteristic downward bowing of the inferior border of the mandible, most apparently visible on radiographs and CT scans. [4]

Histologically, JOF is characterized by the presence of a cellular fibrous stroma, garland-like bony strands, and cement particles. [10] A recent study by El-Mofty identified two histopathological variants, trabecular JOF (TrJOF) and Psammomatoid JOF (PsJOF). [11] The average age of occurrence of TrJOF is considerably younger than PsJOF with the average being eight-and-a half to twelve years as compared to a 16- to 33-year range of PsJOF. With respect to the site, PsJOF occurs overwhelmingly in the sinonsal and orbital bones of the skull, whereas TrJOF, is predominantly a gnathic lesion affecting the jaws with a predilection for the maxilla [12] which was in contrast to the reported case, wherein, the mandible was affected. The presence of proliferating cellular connective tissue with plump cells is suggestive of the aggressive nature of the neoplasm. [1]

Juvenile ossifying fibroma needs to be differentiated from malignant bone tumor and other fibro-osseous lesions. It is important to distinguish JOF from the cemento-ossifying fibroma, which is a benign jaw lesion of odontogenic origin arising from the molar and premolar region of the maxilla and predominately occurs in the mandible; however, cemento-ossifying fibroma occurs more commonly in the second to third decades of life, in contrast to the reported case. Microscopically, the cemento-ossifying fibroma consists of fibroma with dense cellularity and small spherical calcifications - 'cementicles'.

Monostotic fibrous dysplasia occurs more commonly in the maxilla than in the mandible and has a blending border without demarcation and has a more homogenous internal structure in contrast to the reported case, where the mandible was affected and showed flecks of calcification. The fact that fibrous dysplasia is multifocal and occurs in older females, leads to ruling out fibrous dysplasia in the reported case. Cementoblastoma was ruled out as it is fused to the roots of involved tooth. Osteoblastomas are rare in jaws, occur in second and third decades of life, with a male predilection, which is also in contrast to the reported case.

Complete excision of JOF is the treatment of choice as was done in the reported case. Despite its benign features, they can be locally invasive, causing significant morbidity and fatal consequences may be induced by intracranial extension. [6] Radiotherapy seems contraindicated because the tumors are believed to be radio-resistant. It is also contraindicated because of the adverse effects of radiotherapy [13] and also due to the increased incidence of malignant transformation, in the lesion, ranging from 0.4 to 40%. [14]

The clinical course of TrJOF is characterized by infrequent recurrence following conservative excision. Overall, the recurrence rates after resection are reported to range from 30 to 56% and this is probably due to incomplete excision resulting from the infiltrative nature of the tumor borders, more than owing to any intrinsic biological properties of the tumor. [6] Despite the aggressive nature of the lesion and the high rate of recurrence, malignant transformation to sacroma has not been reported. [15] Secondary changes such as aneurysmal bone cysts and hemorrhage are commonly associated with JOF. [16]


   Conclusion Top


Juvenile ossifying fibroma is a benign neoplasm of the bone that has a potential for excessive growth, bone destruction, and recurrence. The rapid growth rate exhibited by this lesion can be quite alarming and makes the clinician suspect malignancy. Hence, the role of the oral and maxillofacial radiologist helps in early recognition and diagnosis, which will help to prevent disability and deformity in children. Nevertheless, long-term clinical and radiological follow-up to rule out the possibility of recurrence and malignant transformation is mandatory.


   Acknowledgment Top


The authors acknowledge the contribution of Dr. Girish Girraddi, Professor and HOD, and faculty from the Department of Oral and Maxillofacial Surgery, G.D.C.R.I., Bangalore, Karnataka, India, for their contribution towards the surgical management of this case.

 
   References Top

1.
Rai S, Kaur M, Goel S, Prabhat M. Trabecuar type of juvenile aggressive ossifying fibroma of the maxilla: Reports of two cases. Contemp Clin Dent 2012;3(Suppl 1):S45-50.  Back to cited text no. 1
    
2.
Aggarwal S, Garg A, Aggarwal A, Ahuja N, Rehman F. Juvenile ossifying fibroma: Psammamatoid variant. Contemp Clin Dent 2012;3:330-3.  Back to cited text no. 2
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Byahatti SM, Nayak R, Nayak R, Jayade B, Hosmani J. Juvenile ossifying fibroma in a juvenile diabetic: A case report with surgical management. World J Dent 2010;1:59-64.  Back to cited text no. 3
    
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Triantafillidou K, Venetis G, Karakinaris G, Iordanidis F. Ossifying fibroma of the jaws: A clinical study of 14 cases and review of the litreature. Oral Surg Oral Med Oral Pathol Oral Radiol 2012;114:193-9.  Back to cited text no. 4
    
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Kumar SM, Elangovan S, Shanmugam S, Srividhya S. Juvenile aggressive ossifying fibroma: A rare case report. J Indian Acad Oral Med Radiol 2011;23:S445-7.  Back to cited text no. 5
    
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Carvalho B, Pontes M, Garcia H, Linhares P, Vaz R. Ossifying fibromas of the craniofacial skeleton. In: Martínez EP, Editor. Histopathology - Reviews and Recent Advances. Rijeka, Croatia: InTech; 2012. p. 121-32.  Back to cited text no. 6
    
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Ravikumar R, Raghavendra K, Kumar S. Aggressive juvenile ossifying fibroma of the anterior mandible. J Dent Sci Res 2011;2:26-34.  Back to cited text no. 7
    
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Chrcanovic BR, Freire-Maia B. An expanded juvenile ossifying fibroma in maxillary sinus: A case report. J Korean Assoc Oral Maxillofac Surg 2011;37:127-32.  Back to cited text no. 8
    
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Wiedenfeld KR, Neville BW, Hutchins AR, Bell RA, Brock TR. Juvenile ossifying fibroma of maxilla in a 6-year-old male: Case report. Pediatric Dent 1995;17:365-7.  Back to cited text no. 9
    
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Keles B, Duran M, Uyar Y, Azimov A, Demirkan A, Esen HH. Juvenile ossifying fibroma of the mandible: A case report. J Oral Maxillofac Res 2010;1:e5.  Back to cited text no. 10
    
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Guruprasad Y, Giraddi G. Juvenile ossifying fibroma of maxilla. J Maxillofac Oral Surg 2010;9:96-8.  Back to cited text no. 11
    
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Urs AB, Kumar P, Arora S, Augustine J. Clinicopathologic and radiologic correlation of ossifying fibroma and juvenile ossifying fibroma - An institutional study of 22 cases. Ann Diagn Pathol 2013;17:198-203.  Back to cited text no. 12
    
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Smith SF, Newman L, Walker DM, Papadopoulos H. Juvenile aggressive psammomatoid ossifying fibroma: An interesting, challenging, and unusual case report and review of the literature. J Oral Maxillofac Surg 2009;67:200-6.  Back to cited text no. 13
    
14.
Guruprasad R, Gupta SD, Dua N, Mehta R. Juvenile psammomatoid ossifying fibroma - A case report. People's Journal of Scientific Research 2011;4:31-4.  Back to cited text no. 14
    
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Nair P, Kumar A, Hegde K, Neelakantan S. Psammomatoid juvenile ossifying fibroma: A case report with literature review. J Indian Acad Oral Med Radiol 2010;22:S53-7.  Back to cited text no. 15
    
16.
Emerson L, Alexander M, Job A. Juvenile trabecular and psammomatoid variant of ossifying fibroma of the maxilla with secondary aneurysmal bone cyst. The Internet Journal of Otorhinolaryngology 2012;14:[about 2 p.]. Available from: http://ispub.com/IJORL/14/1/13975.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]



 

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