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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 7  |  Issue : 1  |  Page : 34-37

Juvenile ossifying fibroma of the mandible: A case report and review


1 Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India
2 Department of Orthodontics and Dentofacial Orthopaedics, Faculty of Dental Sciences, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India

Date of Web Publication11-Jun-2018

Correspondence Address:
Dr. Akhilesh Kumar Singh
Department of Oral and Maxillofacial Surgery, Faculty of Dental Sciences, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jdas.jdas_26_17

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  Abstract 


Juvenile ossifying fibroma is a benign lesion similar to an ossifying fibroma, although more aggressive and of earlier onset. Clinically, it is a slowly growing asymptomatic swelling causing facial asymmetry, but the tumor can progress to a considerable size. Nonaggressive tumors can be managed conservatively, but aggressive lesions may require salvage procedures to prevent recurrence. We present a case of 3-year-old boy with slow growing, firm, nontender swelling of left lower jaw region. The nodular mass obliterated the buccal vestibular region and led to difficulty in mastication and speech. The tumor was treated by en bloc excision through intraoral mandibular vestibular approach.

Keywords: Aggressive, central ossifying fibroma, excision, juvenile, mandible


How to cite this article:
Singh AK, Kumar N, Singh S, Pandey A, Verma V. Juvenile ossifying fibroma of the mandible: A case report and review. J Dent Allied Sci 2018;7:34-7

How to cite this URL:
Singh AK, Kumar N, Singh S, Pandey A, Verma V. Juvenile ossifying fibroma of the mandible: A case report and review. J Dent Allied Sci [serial online] 2018 [cited 2019 Oct 21];7:34-7. Available from: http://www.jdas.in/text.asp?2018/7/1/34/234184




  Introduction Top


Central ossifying fibroma (COF) is a jaw bone fibro-osseous lesion.[1],[2],[3] It is a slow-growing, asymptomatic, well-demarcated, benign lesion in the mandible or maxilla. Sometimes due to the presence of both bone and cementum-like products, these lesions are designated as ossifying fibroma, cement-ossifying fibroma, and cementifying fibroma.[1] Juvenile ossifying fibroma (JOF) is an uncommon, benign, bone-forming neoplasm that is differentiated from other fibro-osseous lesions primarily by its age of onset, clinical presentation, and potential behavior. It occurs most commonly between 5 and 15 years of age and shows male predilection.[4] It may exhibit rapid growth of the involved anatomic site, sometimes resulting in considerable facial disfigurement.[5] Mostly, JOFs arise in the vicinity of the paranasal sinuses.[6] Here, we present a case of JOF of the mandible in a 3-year-old boy child treated by complete surgical excision.


  Case Report Top


A 3-year-old boy reported to our unit with the chief complaint of a painless swelling in the left lower jaw for the past 10 months [Figure 1]. History of present illness as illustrated by the parents emphasized that the patient was apparently asymptomatic 10 months back when he developed swelling which was insidious in onset, gradually progressive and increased over this period. The swelling was not associated with any kind of pain, ulceration or discharge. The medical and dental history was noncontributory.
Figure 1: Preoperative photograph of child showing diffuse swelling over left side of the midface region

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Extraoral examination revealed facial asymmetry due to firm, diffuse, nontender, nonfluctuant or compressible swelling of dimension approximately 3 cm × 4 cm over the left side of the lower face region. Skin color and texture over the swelling appeared to be normal. Intraorally, a firm, diffuse swelling with more buccal than lingual cortical expansion, obliterating the buccal vestibule was present over the retromolar region of the left side of the mandible [Figure 2]. The swelling was not associated with any secondary mucosal changes. Deciduous molar teeth on the left side were lingually displaced. Routine hematological investigations were normal in the limit. Fine-needle aspiration cytology (FNAC) from the swelling was suggestive of ossifying fibroma. Computed tomographic scan revealed an ill-defined expansile lesion of approximate size 18 mm × 15 mm × 24 mm arising from the left retromolar region of the mandible. The lesion showed partially ossified matrix and cortical expansion. Zone of transition was found narrow. No evidence of associated periosteal reaction or extraosseous soft tissue component [Figure 3] and [Figure 4]. A provisional diagnosis of JOF was made after correlating the clinical, radiological, and FNAC findings.
Figure 2: Intraoral photograph showing diffuse swelling over left lower retromolar region obliterating the buccal vestibule

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Figure 3: Coronal computed tomography image showing an ill-defined expansile lesion arising from the left retromolar region with partially ossified matrix and cortical expansion

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Figure 4: Three-dimensional Computed tomographic view showing the lesion over anterior border of ramus with bone resorption surrounding it

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Considering the age of the patient, a conservative approach was planned. Complete surgical excision of the encapsulated tumor mass was performed under general anesthesia through an intra-oral mandibular vestibule approach [Figure 5] and [Figure 6]. The defect created was primarily closed using 3–0 vicryl sutures. The histopathological study of the excised lesion was consistent with the previous reports. The patient is under regular follow-up and has been without any signs of locoregional recurrence for the past 2 years [Figure 7].
Figure 5: Intraoperative photograph showing a firm lesion exposed through vestibular approach

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Figure 6: Excised lesion measuring approximately 3 cm × 4 cm

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Figure 7: Postoperative photograph of the child with bilateral symmetrical face

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  Discussion Top


JOF is a fibro-osseous neoplasm, is an active growing lesion which includes a cell-rich fibrous stroma, containing bands of cellular osteoid without osteoblastic lining, along with trabeculae of more typical woven bone. Sometimes, small foci of giant cells are also present. The lesion is nonencapsulated but well-demarcated from surrounding bone.[7] Approximately 30%–58% recurrence has been reported.[8] Clinically, JOF is a slowly growing asymptomatic swelling causing facial asymmetry, but the tumor can progress to considerable size and behaves, as an aggressive lesion. Occasionally, pain and parasthesia are present. Depending on anatomical site involvement, nasal obstruction, epistaxis, and exophthalmos are observed. Slight male predilection with age ranging from 2 to 15 years.[9] Some cases are part of a syndrome including primary hyperparathyroidism (HPT) (caused by adenoma or carcinoma of the parathyroid) and renal lesions, the so-called “HPT-jaw tumor syndrome.” Diagnosis can be important, because of the possible involvement of other family members and also there is a risk of malignancy.[10],[11] A new tumor suppressor gene (HRPT2) mutation has been found in association with ossifying fibroma; such lesions could arise as a result of haplo insufficiency of the particular gene.[12]

Radiographically, JOF varies from unilocular to multilocular radiolucency with well-defined borders and sometimes opacification depending on the stage and time of radiographic examination. The presence of cortical thinning, perforation, tooth displacement, and root resorption, are suggestive of a feature of aggressive JOF, which is not found in case of COF.[9] The high rate of recurrence of JOF has been attributed to the propensity of JOF to perforate cortical bone. The most important radiological feature of JOF is the presence of circumscribed well-defined border which distinguishes it from fibrous dysplasia.[13] It can be classified into two distinct sub-types, i.e., trabecular and psammomatoid variant.[14] With varied clinical and histological features the maxilla, paranasal sinus, orbit, and fronto-ethmoid bone are the most common sites for the involvement of the JOF. Very less cases of mandibular JOF have been reported in the literature.[15]

Malignant neoplasms that have a tendency to involve the craniofacial skeleton in this age group include osteosarcoma, chondrosarcoma, Ewing's sarcoma, and the African form of Burkitt's lymphoma. Benign neoplastic or developmental lesions are more commonly encountered in the jaws, and although, they may show differing degrees of aggressiveness, they often present with the radiographic characteristics of JOF. Included among these diseases are osteoblastoma, an intraosseous form of fibromatosis known as desmoplastic fibroma, cemento-ossifying fibroma, fibrous dysplasia, central giant cell granuloma, and cherubism. Also occurring in the jaws are the benign odontogenic neoplasms and cysts, which include, in part, ameloblastoma, ameloblastic fibroma, ameloblastic fibro-odontoma, adenomatoid odontogenic tumor, and calcifying odontogenic cyst.[5],[16]

Histopathologically, both sub-types are typically nonencapsulated but well-demarcated from surrounding bone. The neoplasm 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 the multinucleated giant cell are also seen. Whereas psammomatoid pattern forms concentric laminated and spherical ossicles that varies in shape and typically shows basophilic centers with peripheral eosinophilic osteoid rims.[17] There is some degree of histologic overlap between JOF and cemento-ossifying fibroma.[16] In contrast to cemento-ossifying fibroma, which histologically is characterized by uniformity of pattern. The juvenile form could be distinguished from ossifying fibroma by the following features: earlier onset (at childhood or adolescence), locally aggressive growth and osteoid trabeculae on histological examination. JOF is more likely to show markedly heterogeneous morphology.[12] The rapid growth rate often exhibited by these lesions can be quite alarming and cause the clinician to suspect the presence of malignancy. Whereas the treatment for JOF recommended by most investigators is conservative excision or curettage, some lesions may necessitate more aggressive management. Long-standing lesions may show significant cortical destruction and periosteal elevation, which can increase the risk of recurrence.[5],[18] Regular follow-up is necessary to observe the recurrence. In our case, no signs of recurrence have been noticed in the past 2 years.


  Conclusion Top


The overall clinical, imaging and histopathological features are required to label the diagnosis of JOF. Due to unpredictable rapid and progressive growth of some JOF, the management and the prognosis of JOF are uncertain. Nonaggressive JOF should be treated by curettage and local excision. Aggressive JOF requires a complete surgical excision, en bloc or hemimaxillectomy to prevent the chances of any recurrence.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Eversole LR, Leider AS, Nelson K. Ossifying fibroma: A clinicopathologic study of sixty-four cases. Oral Surg Oral Med Oral Pathol 1985;60:505-11.  Back to cited text no. 1
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2.
Waldron CA, Giansanti JS. Benign fibro-osseous lesions of the jaws: A clinical-radiologic histologic review of sixty-five cases. Part I. Fibrous dysplasia. Oral Surg Oral Med Oral Pathol 1973;35:190-201.  Back to cited text no. 2
    
3.
Waldron CA, Giansanti JS. Benign fibro-osseous lesions of the jaws: A clinical-radiologic-histologic review of sixty-five cases. II. Benign fibro-osseous lesions of periodontal ligament origin. Oral Surg Oral Med Oral Pathol 1973;35:340-50.  Back to cited text no. 3
    
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Slootweg PJ, Müller H. Juvenile ossifying fibroma. Report of four cases. J Craniomaxillofac Surg 1990;18:125-9.  Back to cited text no. 4
    
5.
Waldron CA. Fibro-osseous lesions of the jaws. J Oral Maxillofac Surg 1993;51:828-35.  Back to cited text no. 5
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6.
Johnson LC, Yousefi M, Vinh TN, Heffner DK, Hyams VJ, Hartman KS. Juvenile active ossifying fibroma. Its nature, dynamics and origin. Acta Otolaryngol Suppl 1991;488:1-40.  Back to cited text no. 6
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7.
Williams HK, Mangham C, Speight PM. Juvenile ossifying fibroma. An analysis of eight cases and a comparison with other fibro-osseous lesions. J Oral Pathol Med 2000;29:13-8.  Back to cited text no. 7
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Waldron CA. Bone pathology. In: Neville BW, Damm DD, Allen CM, Bouguat JE, editors. Oral and Maxillofacial Pathology. Philadelphia: W.B. Saunders; 1995. p. 443-92.  Back to cited text no. 8
    
9.
Papadaki ME, Troulis MJ, Kaban LB. Advances in diagnosis and management of fibro-osseous lesions. Oral Maxillofac Surg Clin North Am 2005;17:415-34.  Back to cited text no. 9
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Aldred MJ, Talacko AA, Savarirayan R, Murdolo V, Mills AE, Radden BG, et al. Dental findings in a family with hyperparathyroidism-jaw tumor syndrome and a novel HRPT2 gene mutation. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:212-8.  Back to cited text no. 10
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11.
Harimaya A, Tsubota H, Hoki K, Sato J, Kondo A, Yamada T, et al. Ossifying fibroma of the mandible with primary hyperparathyroidism due to non-familial parathyroid adenoma. J Laryngol Otol 2007;121:281-4.  Back to cited text no. 11
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12.
Pimenta FJ, Gontijo Silveira LF, Tavares GC, Silva AC, Perdigão PF, Castro WH, et al. HRPT2 gene alterations in ossifying fibroma of the jaws. Oral Oncol 2006;42:735-9.  Back to cited text no. 12
    
13.
Blaschke DD. Diseases of bone manifested in the jaws. In: Goaz PW, White SC, editors. Oral Radiology Principles and Interpretation. St. Louis: Mosby; 1994. p. 494-512.  Back to cited text no. 13
    
14.
El-Mofty S, Louis ST. Psammomatoid and trabecular juvenile ossifying fibroma of craniofacial skeleton: Two distinct clinic pathologic entities. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;93:296-304.  Back to cited text no. 14
    
15.
Leimola-Virtanen R, Vähätalo K, Syrjänen S. Juvenile active ossifying fibroma of the mandible: A report of 2 cases. J Oral Maxillofac Surg 2001;59:439-44.  Back to cited text no. 15
    
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Slootweg PJ. Maxillofacial fibro-osseous lesions: Classification and differential diagnosis. Semin Diagn Pathol 1996;13:104-12.  Back to cited text no. 16
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17.
Neville BW, Damm D, Allen CM, Bouquot JE. Oral and Maxillofacial Pathology. 3rd ed. Philadelphia: W.B. Saunders; 2005.  Back to cited text no. 17
    
18.
MacIntosh RB. Juvenile ossifying fibroma. Oral Maxillofac Surg Clin North Am 1997;9:713-20.  Back to cited text no. 18
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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