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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 3  |  Issue : 1  |  Page : 16-19

Juvenile trabecular ossifying fibroma of the mandible


Department of Oral Pathology, SCB Dental College, Cuttack, Odisha, India

Date of Web Publication3-May-2019

Correspondence Address:
Dr. Surya Narayan Das
Department of Oral Pathology, SCB Dental College, Cuttack, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/oji.oji_10_19

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  Abstract 


Fibro-osseous lesions are bony lesions where the bone is replaced by fibrous connective tissue and include fibrous dysplasia (FD), cemento-osseous dysplasia, and ossifying fibroma (OF). OF is a benign osteogenic mesenchymal tumor and clinicopathologically divided into juvenile and conventional subtypes. Juvenile OF (JOF) is a rare rapidly growing tumor of the craniofacial bones having aggressive clinical behavior with high recurrence rate. Pathologically, JOF is classified into juvenile trabecular OF (JTOF) and juvenile psammomatoid OF. JTOF microscopically shows proliferation of fibrocellular connective tissue stroma along with immature and woven bony trabeculae with varied histologic features. We report a case of JTOF of the mandible in a 12-year-old male with the initial presentation of painless swelling on the right side lower jaw. Clinicoradiologically, the case was suspected as central giant cell granuloma and odontogenic tumors. He was successfully treated with wide local excision and reconstruction and was on follow-up for 8 months without any recurrence of the disease.

Keywords: Juvenile, mandible, ossifying fibroma, trabecular


How to cite this article:
Fatma S, Das SN, Singh S, Rath R. Juvenile trabecular ossifying fibroma of the mandible. Oncol J India 2019;3:16-9

How to cite this URL:
Fatma S, Das SN, Singh S, Rath R. Juvenile trabecular ossifying fibroma of the mandible. Oncol J India [serial online] 2019 [cited 2019 Sep 21];3:16-9. Available from: http://www.ojionline.org/text.asp?2019/3/1/16/257613




  Introduction Top


Ossifying fibroma (OF) is a rare benign fibro-osseous lesion of the maxillofacial region. It is classified as juvenile and conventional variants, based on the clinical and pathological features.[1] Juvenile OF (JOF) is the invasive type of OF and is rapidly growing with aggressive clinical behavior, and most of the cases occur in children and young adults.[2] Most of the cases occurred in the maxilla. The mandible is a rare site of occurrence for JOF, with only a few cases being reported. Surgery is the mainstay of treatment.[3] Pathologically, JOF is classified into juvenile trabecular OF (JTOF) and juvenile psammomatoid OF (JPOF).[4],[5] Herein, we report a case of JTOF of the mandible in 12-year-old male and treated surgically.


  Case Report Top


A 12-year-old male presented with the complaints of a painless swelling on the right side lower jaw for 3½ months. The swelling was rapidly increasing in size within the last 1 month. On extraoral examination, an oval-shaped swelling was present over the right side lower face of size 6 cm × 5 cm extending to the body of mandible anteriorly, posterior aspect of the mandible posteriorly, level of the left ear lobule superiorly, and lower border of the mandible inferiorly [Figure 1]a. The lesion was hard in consistency with surface ulcerated at the central area. Intraoral examination shows buccal cortical plate expansion without any ulceration, and mouth opening was adequate [Figure 1]b.
Figure 1: An oval-shaped swelling over the right side lower face of size 6 cm × 5 cm on extraoral examination (a) and buccal cortical plate expansion without any ulceration on intraoral examination (b)

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Orthopantomograph revealed a well-defined unilocular expansile radiolucent lesion of size approximately 4.5 cm × 5.5 cm involving the right side angle of the mandible with cortical expansion respect to the posterior and the inferior border of the mandible. There was presence of root resorption of lower second molar [Figure 2]a. Computed tomography (CT) on three-dimensional volume-rendered image and axial section represented a unilocular expansile osteolytic lesion involving the right hemimandible with expansion and destruction of bicortical plate and involvement of the lower border of the mandible. There was absence of any calcification or fluid–fluid levels [Figure 2]b and [Figure 2]c. Fine-needle aspiration cytology was done from the swelling and Nature of the aspirate revealed frank blood. Smear examination of the aspirate revealed cellular necrotic material, few oval cells with eccentric nucleus, few epithelial cells and plenty of lymphocytes and polymorphs.
Figure 2: (a) Orthopantomograph showing a well-defined unilocular expansile radiolucent lesion involving the right side angle of mandible with cortical expansion on (a), and computed tomography on three-dimensional volume-rendered image (b) and axial section (c) showing an unilocular expansile osteolytic lesion involving the right hemimandible with cortical breach without any evidence of calcification or fluid–fluid levels

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With the above clinical, radiological, and cytological findings, differential diagnosis of central giant cell granuloma and odontogenic tumors was made. Incisional biopsy was performed from the lesion and revealed features of JTOF.

The patient underwent wide local excision and reconstruction using costochondral graft [Figure 3]a and [Figure 3]b. On gross examination, the tumor was grayish-color soft to hard in consistency measuring 4.5 cm × 1.8 cm × 0.5 cm [Figure 3]c and [Figure 3]d. Microscopic examination revealed an unencapsulated, fibromyxoid stroma containing spindle and polyhedral cells, with areas exhibiting variable degrees of cellularity and collagenization, immature bony trabeculae with osteoblastic rimming, and aggregates of multinucleated giant cells. In some areas, the osteoid was difficult to distinguish from the stroma. Pseudocystic degeneration and aggregates of hemorrhage were seen [Figure 4]a,[Figure 4]b,[Figure 4]c,[Figure 4]d. Cut section of the mandible was microscopically free of the tumor.
Figure 3: Intraoperative findings showing tumor (a) and reconstruction after removal of the tumor using costochondral graft (b) and gross examination showing grayish-red-colored tumor (c and d)

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Figure 4: (a) Hypercellular fibrous stroma with immature bony trabeculae, pseudocystic degeneration, and areas of hemorrhage (H and E, ×100), (b) woven bone with hypercellular stroma (H and E, ×400), (c) multinucleated giant cells in the background of fibrovascular stroma (H and E, ×400) and (d) garland-like bony strand with entrapped osteoblasts (H and E, ×400)

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The patient was under regular follow-up without any evidence of recurrence for the last 8 months.


  Discussion Top


JOF and other benign fibrous lesions have similar clinical features, imaging characteristics, and pathological features and require a systematic understanding of the disease for accurate diagnosis and a reasonable treatment plan. JOF is distinguished from the conventional OF in regard with patient age, location, and clinical behavior. OF is commonly seen in adults (30–40 years age group), whereas JOF patients are seen in relatively younger age.[6] JOF has no sex predilection and commonly occurs in craniofacial bone involving paranasal sinuses, ethmoid, eyes, and upper jaws. It rarely involves long bones of the body and mandible.[1]

JTOF predominantly affects children and adolescents, with a mean age of 8.5–12 years, whereas JPOF usually affects a wider patient age range with a mean age of 16–33 years.[7] JTOF is commonly seen in the maxilla followed by the mandible, and extragnathic occurrence is rare but JPOF predominantly affects the extragnathic craniofacial bones, i.e., paranasal sinus bone wall, particularly the periorbital, frontal, and ethmoid bones.[5],[6],[7]

Clinically, JOFs are asymptomatic and diagnosed incidentally on radiographic imaging. Displacement of the tooth may be the initial detection and rapid growth leads to expansion of the involved bone resulting facial asymmetry and jaw deformity.[7] Pain and paresthesia are less common. Clinical presentation depends on the site of the lesion and its extension such as exophthalmos, diplopia and other visual symptoms, nasal obstruction, epistaxis, and others.[8]

Radiographically, JOF is a well-demarcated expansile lesion and can be radiolucent, mixed, opaque, and ground glass-like appearance due to the internal components of hard and soft tissues. Most of the cases are unilateral in location, and due to rapid growth, there may be cortical thinning and perforation.[3],[6] Due to the wide association of aneurysmal bone cyst with JOF, CT examination shows fluid level. JPOF usually shows ground-glass appearance, but JTOF shows mixed or radiolucent lesion.[6]

Radiologically, FD, osseous dysplasia (OD), odontoma, and ameloblastoma are the important differential diagnosis of JOF. FD resembles ground-glass appearance like JPOF but lacks clear boundaries involving multiple bones. Whereas, JTOF maintains a well-defined corticated border lacking ground-glass appearance as in our case.[7] OD usually has calcification between the root space with a varied amount of cotton-like calcification involving the bilateral upper and lower jaws. Odontoma is usually a mixed density mass around the roots with clear boundaries and has nonerupted teeth or shadows of teeth of varying sizes. Ameloblastoma usually shows multiple radiolucent regions with discrete, corticated borders exhibiting honeycomb appearance. Our case has unilocular radiolucent appearance of the lesion. Calcifications are absent in our case, which is usually prominent in OD.[6] Rapid growing nature of the tumor along with younger age group of presentation may mimic malignancy, particularly osteosarcoma. Osteosarcoma shows destructive irregular cortical margins invading the periodontal ligament and soft tissues without any corticated boarder, but JTOF has a corticated border.[7]

Histologically, JOF performs heterogeneous morphology having areas of hypercellular fibrous stroma. Bone trabeculae and ossicles are unevenly distributed with possible appearance of bone ropes similar to garland as in our case, and basophilic cementum-like particles and giant cells may be present.[1],[3] JTOF is commonly a well-demarcated but unencapsulated lesion having plump osteoblastic rimming. Foci of hemorrhage and pseudocystic stromal degeneration may be seen along with occasional mitosis.[5],[7]

Pathologically, JPOF, cemento-OF (COF), FD, cemento-OD (COD), and osteoblastoma are the various differential diagnosis for JTOF.[7] Mineralized component in the two patterns of JOF is different. JPOF shows numerous spherical ossicles resembling psammoma bodies in the background of dense cellular fibrous stroma. Whereas, JTOF shows the presence of thin trabeculae and woven bone with coarse lacunae and swollen osteocytes.[9] COF occurs in older-age group of patients. FD has irregular curvilinear shaped bony trabeculae in the background of fibrous stroma but characteristically encompasses osseous cortex. COD has varied histopathological appearance depending on the stage of maturation but has thicker bony trabeculae with less osteoblastic rimming in contrast to JTOF.[7] Osteoblastoma shows the presence of proliferated plump osteoblasts along with dilated blood vessels and multilayered overlapping of osteoblasts and osteoclasts.[10]

As there is no difference between limited surgery and more radical approach in terms of outcome, conservative treatment can be the preferred option. However, JOF has aggressive behavior and high rate of local recurrence (30%–58%) requiring more radical approach. Complete surgical resection should be the preferred options.[1],[9] Our case underwent resection and reconstruction for the mandibular JTOF.


  Conclusions Top


Mandible is a rare site of occurrence for JTOF. Its diagnosis requires combination of clinical, growth pattern, imaging, and pathological features. Early diagnosis of the lesion is important to avoid extensive surgery and its consequence deformity. There is no standard treatment and follow-up schedule for JOF and needs further evaluation.

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.
Seifi S, Foroghi R, Rayyani A, Kiani M, Mohammadi MR. Juvenile trabecular ossifying fibroma – A case report. Indian J Surg Oncol 2018;9:260-4.  Back to cited text no. 1
    
2.
Rao S, Nandeesh BN, Arivazhagan A, Moiyadi AV, Yasha TC. Psammomatoid juvenile ossifying fibroma: Report of three cases with a review of literature. J Pediatr Neurosci 2017;12:363-6.  Back to cited text no. 2
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3.
Khanna J, Ramaswami R. Juvenile ossifying fibroma in the mandible. Ann Maxillofac Surg 2018;8:147-50.  Back to cited text no. 3
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4.
El-Mofty S. Psammomatoid and trabecular juvenile ossifying fibroma of the craniofacial skeleton: Two distinct clinicopathologic entities. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;93:296-304.  Back to cited text no. 4
    
5.
El-Mofty SK, Nelson B, Toyosawa S. Ossifying fibroma. In: El-Naggar AK, Chan JK, Grandis JR, Takata T, Slootweg PJ, editors. WHO Classification of Head and Neck Tumours. 4th ed. Lyon: IARC Press; 2017. p. 251-2.  Back to cited text no. 5
    
6.
Han J, Hu L, Zhang C, Yang X, Tian Z, Wang Y, et al. Juvenile ossifying fibroma of the jaw: A retrospective study of 15 cases. Int J Oral Maxillofac Surg 2016;45:368-76.  Back to cited text no. 6
    
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Sultan AS, Schwartz MK, Caccamese JF Jr., Papadimitriou JC, Basile J, Foss RD, et al. Juvenile trabecular ossifying fibroma. Head Neck Pathol 2018;12:567-71.  Back to cited text no. 7
    
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Malaviya P, Choudhary S, Gupta S, Toshniwal OD. Trabecular variant: A rare entity of juvenile ossifying fibroma of the mandible. Contemp Clin Dent 2017;8:179-81.  Back to cited text no. 8
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Aslan F, Yazici H, Altun E. Psammomatoid variant of juvenile ossifying fibroma. Indian J Pathol Microbiol 2018;61:443-5.  Back to cited text no. 9
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Ranganath K, Kamath SM, Munoyath SK, Nandini HV. Juvenile psammomatoid ossifying fibroma of maxillary sinus: Case report with review of literature. J Maxillofac Oral Surg 2014;13:109-14.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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