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 Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 4  |  Issue : 2  |  Page : 78-81

Psammomatoid juvenile ossifying fibroma of maxilla in a 45-year-old female patient


Department of Oral and Maxillofacial Pathology, Guru Nanak Institute of Dental Sciences and Research, Panihati, Kolkata, West Bengal, India

Date of Web Publication22-Oct-2013

Correspondence Address:
Sanchita Kundu
Guru Nanak Institute of Dental Sciences and Research, Panihati, Kolkata - 700 114, West Bengal
India
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DOI: 10.4103/0976-433X.120183

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  Abstract 

Juvenile ossifying fibroma (JOF) is an uncommon, benign, yet aggressive fibro-osseous lesion of the jaws commonly occurring in children and young adults and affects equally both males and females. Maxilla is affected more often than mandible. Two microscopically distinct types of JOF, trabecular and psammomatoid varieties have been described, of which latter being more common. A clinical case of psammomatoid variant of JOF (PsJOF) involving the right side of maxilla of a 45-year-old female patient is discussed herewith, encompassing a thorough knowledge of clinical, radiological, and histopathological features.

Keywords: Aggressive, juvenile, ossifying fibroma, psammoma-like bodies


How to cite this article:
Kundu S, Pal M, Ghosh S, Chatterjee RP. Psammomatoid juvenile ossifying fibroma of maxilla in a 45-year-old female patient. SRM J Res Dent Sci 2013;4:78-81

How to cite this URL:
Kundu S, Pal M, Ghosh S, Chatterjee RP. Psammomatoid juvenile ossifying fibroma of maxilla in a 45-year-old female patient. SRM J Res Dent Sci [serial online] 2013 [cited 2019 Nov 13];4:78-81. Available from: http://www.srmjrds.in/text.asp?2013/4/2/78/120183


  Introduction Top


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 World Health Organization (WHO). [1] Although JOF was described in the past as a variant of ossifying fibroma, more recently it has been segregated into two distinct sub types:

1) Trabecular variant: WHO type (TJOF), and 2) Psammomatoid variant (PsJOF) with varied clinical and histological features. [2]

PsJOF is an aggressive lesion with a predilection for occurrence in children in the age range of 5-15 years; there are reports where the patient age ranged from 3 months-72 years. [3],[4] There is no significant sex predilection for either variant. Approximately, 75% of PsJOFs develop in the orbit, paranasal sinuses, and calvaria; whereas, only about 25% of all cases involve the maxilla or mandible. [5] In contrast, about 95% of the documented cases of TJOF have developed within the jawbones, with maxillary lesions occurring more frequently. It has been proposed that the mesenchymal cells of the periodontal membrane serve as multipotential precursor cells capable of differentiation into cementum, osteoid or fibrous tissue, and give rise to a spectrum of fibro-osseous lesions. [2],[6] According to some investigators, these lesions perhaps originated from maldevelopment of the tissue, generating the bony septa between the roots of molar teeth. [6],[7] The presence of nonrandom chromosomal breakpoints at Xq26 and 2q33 resulting in (x, 2) translocation have also been reported. [2]

The clinical signs and symptoms are related to the anatomic site of involvement. Clinically in most instances, JOF presents as a slow-growing asymptomatic swelling causing facial asymmetry, but the tumor can progress to considerable size and behaves as an aggressive lesion. Less commonly, pain and paresthesia are noted. Depending upon the anatomical site of involvement, nasal obstruction, epistaxis, and exopthalmos are observed. [5],[8],[9] Radiographically, the internal structure can be radiolucent or mixed or radiopaque or having a 'ground-glass' appearance depending upon the degree of calcification. Root displacement is common and resorption, though rare, can occur. The lesion can cause expansion as well as perforation of the cortical plates. JOF is not capsulated but is separated from surrounding bone by a radiopaque border, and this finding can help in differentiating it from fibrous dysplasia. Maxillary tumors often fill and obliterate the maxillary sinus, whereas mandibular tumors usually involve the ramus and angle. [2],[3],[4],[5],[8]

Microscopically PsJOF is slightly more cellular than TrJOF. [5] The lesion is characterized by a fibroblastic stroma containing small ossicles, resembling psammoma bodies. These ossicles vary in shape and typically have basophilic centers with peripheral eosinophilic osteoid rims. A peripheral brush border blending into the surrounding stroma is also noted in many of the ossicles. These unique spherical structures were first described as "psammoma-like" bodies by Neville et al. [2] The word "psammoma" is derived from a Greek word "psammos" meaning "sand". [6] The stroma varies from being loose and fibroblastic to intensely cellular with minimum intervening collagen along with occasional presence of giant cells. [2],[5],[10] The neoplasm is highly aggressive in nature because of its high recurrence rate (30-58%) [4],[6],[9] and treated like a locally aggressive neoplasm, very much like an ameloblastoma. Surgical resection, rather than conservative curettage, is therefore the preferred line of treatment. [3],[9]

Based on the above clinicopathological, radiological, and histopathological knowledge, a case of PsJOF involving the right side of maxilla is discussed herewith.


  Case Report Top


A 45-year-old female patient reported to the Oral and Maxillofacial Pathology Department with a chief complaint of swelling involving the right side of face for about 2-3 years. Extraorally, there was presence of a large, hard, nontender swelling with well-defined margins involving the middle third of right side of face. Overlying skin appeared normal [Figure 1] and [Figure 2]. Intraoral examination revealed the presence of a large bony hard, well-defined, nontender central swelling involving the maxilla in relation to upper right second premolar to second molar region associated with expansion of buccal cortical plate. Displacement of upper right second premolar, first and second molars were also noted [Figure 3]. Orthopantomogram (OPG), paranasal sinus (PNS) view, and computed tomography (CT scan revealed the presence of a large well-defined central jaw lesion showing mixed radiographic changes/ground-glass appearance in relation to upper right second premolar to third molar region characterized by involvement of the maxillary antrum, displacement and mild root resorption of upper right second molar [Figure 4], [Figure 5], and [Figure 6].
Figure 1: Extraoral front profile photograph showing the presence of a medium-sized diffused swelling involving the right middle and lower one-third of face being covered by normal appearing skin of the patient in the case report

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Figure 2: Extraoral lateral profile photograph showing the presence of a medium sized diffused swelling involving the right middle and lower one-third of face being covered by normal appearing skin of the patient in the case report

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Figure 3: Intraoral photograph showing a large, bony, hard, well-defined, nontendered swelling involving the maxilla in relation to upper right second molar to second premolar region associated with expansion of buccal cortical plate. Displacement of upper right second premolar and first and second molars were also noted of the patient in the case report

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Figure 4: Panoramic radiograph showing large, well-defined, central jaw lesion characterized by mixed radiographic changes/ground-glass appearance in relation to upper right third molar to second premolar region along with involvement of the right maxillary antrum

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Figure 5: Paranasal sinus view (PNS) showing the involvement of the right maxillary antrum

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Figure 6: Computed tomography (CT) scan revealing the presence of a large, well-defined, radioopaque lesion in relation to right first premolar to second molar region associated with expansion of the buccal cortical plate, involvement of the right maxillary antrum and displacement of upper right second molar region

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Based on above clinical and radiological findings, an incisional biopsy was performed from a representative site of the lesion. The light microscopic features revealed the presence of cellular fibroblastic connective tissue stroma containing multiple spherical and curved ossicles resembling psammmoma bodies. Periphery of the ossicles showed a brush border which was blending into the surrounding connective tissue stroma [Figure 7], [Figure 8], and [Figure 9].
Figure 7: Low power photomicrograph showing the presence of cellular fibroblastic connective tissue stroma containing multiple spherical and curved ossicles

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Figure 8: High power photomicrograph showing the presence of "psammmoma" body

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Figure 9: High power photomicrograph showing the presence of "psammmoma" body, Periphery of whichshowed a brush border blended into the surrounding connective tissue stroma

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After considering these microscopic features, a diagnosis of PsJOF was made and patient was referred to Department of Oral and Maxillofacial surgery for further treatment.


  Discussion Top


The spectrum of fibro-osseous lesions includes a variety of developmental, reactive or dysplastic lesions and neoplastic entities. JOFs are benign, potentially aggressive fibro-osseous lesions of the craniofacial bones. In addition to its aggressive behavior, it has a strong tendency to recur. [10] The most characteristic feature of JOF, as the name suggests, is its higher incidence in children and young adults. However, its occurrence in the older age group has also been reported. [3],[4] In the present case, the patient was a 45-year-old female, having a well-defined swelling involving the right side of maxilla leading to obvious facial asymmetry and the presence of a large bony hard, well-defined, nontender central swelling involving the maxilla in relation to upper right premolar-molar region associated with expansion of buccal cortical plate intraorally associated with displacement of upper right second premolar and first and second molars. These clinical findings are consistent with the clinical observations reported by the authors of different studies. [5],[8],[9],[10]

The conventional radiological findings along with CT scan usually include radiolucent/mixed/radiopaque central jaw lesion sometimes having a typical 'ground-glass' appearance, depending on the degree of calcification associated with expansion of the cortical plates. Root displacement is common and resorption, though rare, can occur. The lesion is also separated from surrounding bone by a radiopaque border. There may be involvement of maxillary antrum in case of maxillary lesion. The panoramic view as well as the computed tomographic view of the present case was thus very characteristic of JOF. [2],[3],[4],[5],[8]

The light microscopic features of PsJOF usually reveal cellular fibroblastic connective tissue stroma containing spherical and curved ossicles resembling "psammoma" bodies; possess a dark rim of crystals from which small spicules and needle-like crystalloids project toward the periphery. Presence of spherical and curved ossicles resembling "psammoma" bodies in a cellular fibroblastic connective tissue stroma along with peripherally radiating brush borders which was blending into the surrounding stroma in the present case were strongly mimicking the features of PsJOF. [2],[5],[6],[10]

The entire gamut of discussion involving clinical imaging and microscopic features thus clearly explains the aggressive nature of PsJOF.



The juvenile forms of ossifying fibromas are uncommon, but they need to be recognized and managed appropriately because of their distinctive clinical behavior. The rapid growth rate often exhibited by these lesions can be quite alarming and cause the clinician to suspect the presence of a more aggressive lesion.

 
  References Top

1.Kramer IR. The World Health Organisation: Histological typing of odontogenic tumors: An introduction to the second edition. J Dent Assoc S Afr 1992;47:208-10.  Back to cited text no. 1
    
2.Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and Maxillofacial Pathology. 3 rd ed. India: Elsevier Publishers; 2009. p. 648-50.  Back to cited text no. 2
    
3.Thankappan S, Nair S, Thomas V, Sharafudeen KP. Psammomatoid and trabecular variants of juvenile ossifying fibroma - two case reports. Indian J Radiol Imaging 2009;19:116-9.  Back to cited text no. 3
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4.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. 4
    
5.Malathi N, Radhika T, Thamizhchelvan H, Ravindran C, Ramkumar S, Giri G, et al. Psammomatoid juvenile ossifying fibroma of the jaws. J Oral Maxillofac Pathol 2011;15:326-9  Back to cited text no. 5
    
6.Alper S. Juvenile ossifying fibroma. Report of a case. Gazi Med J 2001;12:67-71.  Back to cited text no. 6
    
7.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. 7
    
8.Offiah C, Hall E. Case of the month. The rapidly enlarging chin mass. Br J Radiol 2005;78:175-6.  Back to cited text no. 8
    
9.Noffke CE. Juvenile ossifying fibroma of the mandible. An 8 year radiological follow-up. Dentomaxillofac Radiol 1998;27:363-6.  Back to cited text no. 9
    
10.Solomon MC, Khandelwal S, AR, Carnelio S. Psammomatoid juvenile ossifying fibroma of the mandible - A histochemical insight! Internet J Dent Sci 2009;7.2  Back to cited text no. 10
    


    Figures

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



 

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