Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 9  |  Issue : 2  |  Page : 114-117

Florid expansile cemento-osseous dysplasia of the jaws: Cone beam computed tomography study and review of the literature


1 Division of Oral & Maxillofacial Radiology, Department of Diagnostic Sciences, Rutgers School of Dental Medicine, Newark, NJ, USA
2 Division of Oral & Maxillofacial Radiology, Department of Oral Medicine, University of Pennsylvania School of Dental Medicine, Philadelphia, PA, USA

Date of Web Publication8-Jan-2018

Correspondence Address:
Dr. Steven R Singer
Room D-885A, Rutgers School of Dental Medicine, 110 Bergen Street, Newark, NJ
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jofs.jofs_82_17

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  Abstract 


An interesting case of florid expansile cemento-osseous dysplasia (FECOD) of the maxilla and mandible in a 36-year-old female is being presented. The patient presented for consultation for orthognathic surgery and was unaware of these lesions. Upon clinical and radiographic examination, including cone beam computed tomography, massive cemento-osseous lesions in all quadrants were observed. The radiographic appearance of the lesions was consistent with cemento-osseous dysplasia (COD), the key difference being extreme expansion. Expansion, although not a new phenomenon, is present in all quadrants. These radiographic features suggest a variation of florid cemento-osseous dysplasia and is more aptly termed FECOD. This name is proposed for its diagnostic relevance based on the radiographic features. As with similar CODs, unless the lesions are disfiguring, conservative management is the preferred approach. Biopsy was not indicated unless there are additional associated complications. A detailed review of the pertinent literature was undertaken.

Keywords: CBCT, cemento-osseous dysplasias, differential diagnosis, expansion, jaws


How to cite this article:
Singer SR, Creanga AG, Vyas R, Mupparapu M. Florid expansile cemento-osseous dysplasia of the jaws: Cone beam computed tomography study and review of the literature. J Orofac Sci 2017;9:114-7

How to cite this URL:
Singer SR, Creanga AG, Vyas R, Mupparapu M. Florid expansile cemento-osseous dysplasia of the jaws: Cone beam computed tomography study and review of the literature. J Orofac Sci [serial online] 2017 [cited 2019 Sep 16];9:114-7. Available from: http://www.jofs.in/text.asp?2017/9/2/114/222390




  Introduction Top


Cemento-osseous dysplasias (COD) are benign fibro-osseous (BFO) lesions often seen in the tooth-bearing areas in women of African descent. Cancellous bone is replaced with dense, acellular amorphous bone in a background of fibrous connective tissue.[1] When these lesions cause expansion of the jaws, they were named expansive osseous dysplasia (EOD), a name proposed by Noffke and Raubenheimer in 2011.[2] EOD falls into a continuum of BFO lesions that include focal osseous dysplasia (FOD) and periapical cemento-osseous dysplasias (PCD).[3] Although the term EOD fits the descriptions for PCD and florid cemento-osseous dysplasia (FCOD), when the lesions are florid and expansile, the term florid expansile cemento-osseous dysplasia (FECOD) may be more appropriate. Hence, we propose the use of this name for generalized or florid cases of expansile CODs that have overt clinical features of marked jaw expansions. These lesions have similar histopathology, varying only in location, number, and extent, as well as effects on the structures adjacent to the lesions. The coalescence of expanded lesions may also be significant in its differentiation from other similar BFO lesions. Careful radiographic diagnosis is important, because biopsy and surgery are typically undesirable and might lead to complications including poor healing and osteomyelitis.[4],[5]


  Case Report Top


A 38-year-old female of African descent presented for evaluation for orthognathic surgery with no relevant medical history. There were no complaints of pain. Subsequent to anamnesis and a clinical examination, a cone beam computed tomography (CBCT) examination of the maxilla and mandible was ordered. The scan demonstrated multiple mixed density lesions with irregular sclerotic borders and amorphous internal high-density areas in the tooth-bearing areas of the jaws. The hypodense areas of the lesions were also noted and may indicate the areas of resorption and resemble simple bone cysts from a histopathologic standpoint. The lesions were expansile, causing expansion and thinning of the cortices of both the jaws without apparent perforation, superior displacement of the cortical floor of the antra, and the inferior displacement of the mandibular canal. Although the lesions may have originated independently, they appear to have coalesced in each jaw as they expanded.

Panoramic reconstruction from a large volume CBCT shows the mixed density expansile lesions in all quadrants in the alveolar portions of the jaws and extending to the basal bone regions and thinning the superior cortical outlines [Figure 1]. The multiplanar reconstructions further demonstrated the expansion. Although, on occasion, the borders are not quite visibly demarcated, for the most part, the borders are very distinct [Figure 2]. Aside from the slight displacement and supra-eruption due to the lack of opposing teeth, the effect of the lesions on the dentition appears to be negligible. The dentition was unrestored, free of radiographically apparent carious lesions, and supported by the adequate levels of periodontal bone [Figure 3]. The temporomandibular joints appear unaffected bilaterally. It was recommended that the lesions be observed without biopsy and that any orthognathic surgical interventions be performed beyond the borders of the lesions, due to the inherently poor vascularity of the BFO lesions and the concomitant risk of osteomyelitis. The findings were confirmed when reviewed in the sagittal, axial, and coronal sections of the maxilla and mandible as well as the maximum intensity projection (MIP) views [Figure 4].
Figure 1: CBCT panoramic reconstruction demonstrating the mixed density lesions in the tooth-bearing areas of the maxilla and mandible

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Figure 2: CBCT axial projection of the mandible demonstrating expansion and thinning of the cortices. The teeth appear to be unaffected

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Figure 3: CBCT coronal projection demonstrating expansion of the maxilla and mandible, as well as the displacement of the floor of the left maxillary sinus

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Figure 4: Midsagittal projection. The mixed density lesions can be seen in both the CBCT maxilla and mandible. The lamina dura of the teeth is intact

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  Discussion Top


Although the osseous dysplasias have been reported extensively in the literature, this case represents a unique variation with a combination of features seen in other osseous dysplasias, such as FCOD and FOD.[6] Bulut et al. discussed a case of EOD that they have studied where a 24-year-old female patient was referred with a chief complaint of left mandibular swelling that was firm, non-fluctuant, and has normal epithelial mucosa over the expansile area. A panoramic radiography followed by multi-detector computed tomography showed an expansile lesion that was well defined and contained mixed density areas. The expansile lesion was of 2 years duration, and it was reported that it dislodged a removable prosthesis slowly, until it no longer fit the patient. An excisional biopsy was performed, removing the entire mixed density lesion under local anesthesia. Histopathology confirmed the diagnosis of FCOD.[7] The cases of FCOD that can be expansile and deforming can be categorized as EODs. The authors did not report any postsurgical complications such as infection or osteomyelitis, although they should be expected.[7]

Abdelsayed et al. presented three cases with similar radiographic appearance to the present case.[8] Gigantiform cementoblastoma was described as expansile lesions that are multifocal, bilateral, and involving both jaws. Recurrence was reported when surgical excision was attempted. This paper also reported a female predilection for this condition, based on previously reported cases and suggested that is familial, transmitted as an autosomal dominant disorder.

On the basis of the clinical and radiographic features of the present case, the lesions were diagnosed as FECOD with associated simple bone cysts. The presence of the lesions throughout the tooth-bearing areas of the alveolar bone is certainly consistent with the descriptor “florid.” Since biopsy is undesirable, clinical and radiographic follow-up is essential.

The CODs should be differentiated from Paget’s disease, osteomyelitis, and osteosarcoma. While age, symptoms, and radiographic characteristics may favor a specific diagnosis, biopsy may become necessary when more aggressive lesions, including cemento-ossifying fibroma (COF) and malignancy, cannot be excluded from the diagnosis based on less invasive diagnostic methodology. In the present case, Paget’s disease can be discounted by the patient’s age (36 years), and the presence of the lesions in both jaws [Figure 5]. Additionally, Paget’s disease would be seen in the basal bone of the mandible, whereas the lesions of the present case are confined to the tooth-bearing areas of the alveolus.[9] Alkaline phosphatase level may also be checked for an increase where Paget’s disease is suspected. Paget’s disease also will cause effacement of the lamina dura surrounding the teeth in the affected area.[10] The lack of pain and other inflammatory changes, as well as sequestrum formation, would also tend to minimize the possibility of these lesions being osteomyelitis. COF is a frequent manifestation of the hyperparathyroidism-jaw tumor syndrome, caused by germline mutations in the HRPT2 gene.[11] COF tends to occur singularly in either jaw. Additionally, COF is usually more rapidly expanding and is often disfiguring once it reaches a noticeable size. Further, the easily determinable blood hormone levels are definitely altered in a pathologic endocrine environment such as hyperparathyroidism. Osteosarcoma often presents with paresthesia and demonstrates the destruction of the cortices, rather than the thinning and expansion demonstrated in the lesions of the present case. The lack of resorption or displacement of the roots of the adjacent teeth also ends to discount the possibility of osteosarcoma. Additionally, the lack of periosteal reaction would add to the dismissal of infectious, inflammatory, or malignant entities. Fibrous dysplasia (FD) was also discounted in the differential diagnosis due to patient’s age, and the bilateral presentation of the lesions. Further, the borders of FD and the surrounding bone tend to present radiographically as a gradual transition. As in most cases when biopsy is not performed, regular clinical and radiographic follow-up is mandatory to monitor any changes in clinical and radiographic appearance and behavior.
Figure 5: Right and left lateral maximum intensity projections CBCT demonstrating the extent of FECOD lesions

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Genetic analysis may also help to distinguish the lesions of FCOD from FD. It is established that FD is caused by the mutations of the Arg201 codon of the GNAS gene.[12]

Although the significant expansion of the lesions is somewhat unique, the clinical and radiographic data, including associated low-density areas resembling simple bone cysts, point to FCOD and, therefore, FECOD as a reasonable diagnosis.[2],[13],[14] Clinical and radiographic follow-up have been recommended to the patient.

The crucial diagnostic aspect of the CODs is the differentiation from aggressive lesions requiring intervention. CODs are poorly vascularized and susceptible to infection, so biopsy should be avoided. The expansile nature of the lesions in this case mimic the lesions mentioned in the differential. Ongoing surveillance is indicated for the clinical confirmation of the diagnosis. CBCT is an ideal imaging modality to visualize the three-dimensional extent.

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.
Gonçalves M, Píspico R, Alves FD, Lugão CE, Gonçalves A. Clinical, radiographic, biochemical and histological findings of florid cemento-osseous dysplasia and report of a case. Braz Dent J 2005;16:247-50.  Back to cited text no. 1
    
2.
Noffke CE, Raubenheimer EJ. Expansive osseous dysplasia: Report of 9 lesions in an African population sample and a review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011;111:e35-41.  Back to cited text no. 2
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3.
Sarmento DJ, Monteiro BV, de Medeiros AM, da Silveira EJ. Severe florid cemento-osseous dysplasia: A case report treated conservatively and literature review. Oral Maxillofac Surg 2013;17:43-6.  Back to cited text no. 3
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4.
White SC, Pharoah MJ. Other bone diseases. In: White SC, Pharoah MJ, editors. Oral Radiology: Principles and Interpretation. 7th ed. St. Louis: Elsevier; 2014. p. 411-3.  Back to cited text no. 4
    
5.
MacDonald-Jankowski DS. Florid cemento-osseous dysplasia: A systematic review. Dentomaxillofac Radiol 2003;32:141-9.  Back to cited text no. 5
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6.
Kim JH, Song BC, Kim SH, Park YS. Clinical, radiographic, and histological findings of florid cemento-osseous dysplasia: A case report. Imaging Sci Dent 2011;41:139-42.  Back to cited text no. 6
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7.
Bulut EU, Acikgoz A, Ozan B, Zengin AZ, Gunhan O. Expansive focal cemento-osseous dysplasia. J Contemp Dent Pract 2012;13:115-8.  Back to cited text no. 7
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8.
Abdelsayed RA, Eversole LR, Singh BS, Scarbrough FE. Gigantiform cementoma: Clinicopathologic presentation of 3 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;91:438-44.  Back to cited text no. 8
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MacDonald-Jankowski DS. Gigantiform cementoma occurring in two populations, London and Hong Kong. Clin Radiol 1992;45:316-8.  Back to cited text no. 9
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10.
Yildirim E, Bağlar S, Ciftci ME, Ozcan E. Florid cemento-osseous dysplasia: A rare case report evaluated with cone-beam computed tomography. J Oral Maxillofac Pathol 2016;20:329.  Back to cited text no. 10
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11.
Carpten JD, Robbins CM, Villablanca A, Forsberg L, Presciuttini S, Bailey-Wilson J et al. HRPT2, encoding parafibromin, is mutated in hyperparathyroidism-jaw tumor syndrome. Nat Genet 2002;32:676-80.  Back to cited text no. 11
    
12.
Patel MM, Wilkey JF, Abdelsayed R, D’Silva NJ, Malchoff C, Mallya SM. Analysis of GNAS mutations in cemento-ossifying fibromas and cemento-osseous dysplasias of the jaws. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:739-43.  Back to cited text no. 12
    
13.
Raubenheimer EJ, Noffke CE, Boy SC. Osseous dysplasia with gross jaw expansion: A review of 18 lesions. Head Neck Pathol 2016;10:437-43.  Back to cited text no. 13
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MacDonald-Jankowski DS. Fibro-osseous lesions of the face and jaws. Clin Radiol 2004;59:11–25. [Erratum in: Clin Radiol 2009;64:107].  Back to cited text no. 14
    


    Figures

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


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