Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 7  |  Issue : 4  |  Page : 508-512  

Calcifying odontogenic cyst of anterior maxilla with complex odontoma


1 Department of Oral and Maxillofacial Surgery, AME'S Dental College Hospital and Research Centre, Raichur, Karnataka, India
2 Department of Pedodontics and Preventive Dentistry, AME'S Dental College Hospital and Research Centre, Raichur, Karnataka, India
3 Department of Oral and Maxillofacial Pathology, AME'S Dental College Hospital and Research Centre, Raichur, Karnataka, India

Date of Web Publication25-Jun-2014

Correspondence Address:
Yadavalli Guruprasad
Department of Oral & Maxillofacial Surgery, AME'S Dental College Hospital & Research Centre, Raichur - 584 103, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.135290

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  Abstract 

Calcifying odontogenic cyst (COC) is an unusual and unique lesion with characteristics of a solid neoplasm and of a cyst. It shows considerable amount of histopathological diversity, with variable clinical behavior such as cystic, neoplastic and infiltrating malignant behavior. It is a rare developmental odontogenic cyst with notable presence of histopathological features, which include a cystic lining demonstrating characteristic "Ghost" epithelial cells with a propensity to calcify. Odontomes are best known as hamartomatous benign tumors rather than true neoplasms, arising from odontogenic tissues. Histologically, they are classified as compound and complex variety. We report a rare case of COC of anterior maxilla with complex odontome in a 12-year-old female child.

Keywords: Calcifying odontogenic cyst, complex odontoma, maxilla


How to cite this article:
Guruprasad Y, Chauhan DS, Kumar V, Hunsagi P. Calcifying odontogenic cyst of anterior maxilla with complex odontoma. Med J DY Patil Univ 2014;7:508-12

How to cite this URL:
Guruprasad Y, Chauhan DS, Kumar V, Hunsagi P. Calcifying odontogenic cyst of anterior maxilla with complex odontoma. Med J DY Patil Univ [serial online] 2014 [cited 2019 Aug 20];7:508-12. Available from: http://www.mjdrdypu.org/text.asp?2014/7/4/508/135290


  Introduction Top


True bone cysts are often encountered in facial bones due to the presence of embryonic epithelial rests in these bones. A majority of them are remnants of odontogenic apparatus. The calcifying odontogenic cyst (COC) is a developmental odontogenic cyst and its occurrence constitutes about 0.3-0.8% of all odontogenic cysts. Calcifying odontogenic cyst is a rare lesion of the jaws first described as a distinct entity by Gorlin et al., in 1962. [1] The condition is also referred as Gorlin's cyst, keratinizing ameloblastoma or melanotic ameloblastic odontoma. COC is often referred as an asymptomatic slow growing swelling of the jaws. It is a well-circumscribed, solid or cystic lesion derived from odontogenic epithelium, which develops from reduced enamel epithelium or remnants of odontogenic epithelium in the follicle, gingival tissue or bone, but contains "ghost cells" and spherical calcifications. It is considered a unique entity with both cystic and neoplastic behavior. [2],[3] Odontomes are best known as hamartomatous benign tumors rather than true neoplasms, arising from odontogenic tissues. Histologically, they are classified as compound and complex variety. Complex odontoma is a mass of disorganized dental tissues. Clinically, they are classified as intraosseous (central), peripheral (soft-tissue or extra-osseous) and erupted odontomes. The central (intraosseous) odontomes are common representing 51%, occurring in anterior maxilla (compound odontoma) followed by mandibular molar region (complex odontoma). [4] We report a rare case of COC associated with complex odontoma in a 12-year-old female child with emphasis on its clinical features, diagnosis and surgical management.


  Case Report Top


A 12-year-old female patient came with a chief complaint of asymptomatic swelling in the left upper jaw since 6 months. The lesion had been slowly increasing in size since it was first noticed. Extraorally the lesion was present on the anterior maxilla around 3 cm × 3.5 cm in size from the midline of the upper lip to the corner of the mouth thus displacing the left ala of the nose and obliterating the nasolabial fold. The lesion was extending from the left central incisor to first premolar on the same side intraorally and soft in consistency on palpation [Figure 1] and [Figure 2]. A panoramic radiograph showed a well-circumscribed radiolucency in relation to left upper anterior teeth and canine tooth causing root resorption and displacing roots of incisors [Figure 3]. Maxillary occlusal radiograph showing well defined unilocular radiolucency in relation to left anterior and canine teeth along with the odontome [Figure 4]. Computed tomography scan coronal and axial section revealed well-circumscribed radiolucency in relation to left anterior and canine teeth along with the odontome [Figure 5] and [Figure 6]. Fine needle aspiration cytology of the lesion was performed, which yielded straw colored fluid and a provisional diagnosis of dentigerous cyst was made. Patient was posted for surgical exploration under general anesthesia and the lesion was enucleated using intraoral vestibular approach and the entire lesion was excised along with the odontome [Figure 7] and [Figure 8]. The cavity was closed primarily after aggressive curettage. The excised tissue was sent for histopathological examination, which revealed presence of cystic space lined by odontogenic epithelium with ghost cells suggestive of Type 1 COC and the decalcified section showed haphazard arrangement of enamel, dentin, cementum and pulp confirming it as complex odontoma. There were no inductive changes like presence of dentinoid like material in the connective tissue capsule [Figure 9], [Figure 10], [Figure 11]. Post-operative follow-up was done for 1 year and no recurrence was observed.
Figure 1: Frontal view photograph showing slight obvious swelling on the left anterior maxillary region obliterating nasolabial fold

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Figure 2: Submental view photograph showing obvious swelling on the left anterior maxillary region obliterating nasolabial fold and displacing the left ala of the nose

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Figure 3: Orthopantomogram showing unilocular radiolucent lesion in relation to left maxillary anterior and canine teeth thus displacing the involved teeth

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Figure 4: Maxillary occlusal radiograph showing well defined unilocular radiolucency in relation to left anterior and canine teeth. Note the arrows showing the odontome

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Figure 5: Graphical illustration showing the cyst arising from the odontome. Note the shaded area a and b showing the odontome and cystic lining

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Figure 6: Computed tomography scan coronal view showing well defined unilocular radiolucency in relation to left anterior and canine teeth along with the odontome

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Figure 7: Computed tomography scan axial view showing well defined unilocular radiolucency in relation to left anterior and canine teeth along with the odontome

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Figure 8: Intraoperative photograph showing the exposed cystic lining using the vestibular incision

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Figure 9: Excised specimen showing cystic lining along with the odontome. Note the arrow showing the odontome

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Figure 10: Histopathological picture showing cystic space lined by odontogenic epithelium and ghost cells (H and E, ×400)

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Figure 11: Decalcified section of showing disorganized enamel, dentin and cemental matrix confi rming the diagnosis of complex odontoma (H and E, 400)

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  Discussion Top


The COC is known to involve mandible and maxilla with equal frequency. The age of occurrence of the cyst has been reported to vary from 3 years to 80 years with definite peaking in the second decade. [1],[3],[4] The cyst is usually asymptomatic unless secondarily infected. Some cases were reported where the cyst concomitantly occurred with other odontogenic lesions. [2],[4] Praetorius [3] described four types of conjunctional lesions with the cyst: Namely, dentine producing ameloblastoma, odontoameloblastoma, ameloblastic fibro-odontoma and complex odontoma. The presence of ghost cells characterizes the histological appearance of the lesion. Histologically there are 3 types of COC, Type 1: Simple monocystic type with presence of ghost cells with or without dentinoid calcified tissue, Type 2: Formation of calcified tissues in the lumen of the cyst wall, Type 3: Ameloblast like proliferation in the connective tissue and lumen of the cyst may be seen.

The calcifications, if present, will appear scattered radiopaque flakes in the radiograph. The other conditions which may simulate this radiographic appearance are the Pindborg tumor, ameloblastic fibro-odontoma and adenomatoid odontogenic tumor. COC may occur in association with other odontogenic tumors, the most common of these is the odontoma. Hirshberg et al. [4] reveals 52 cases of COC associated with odontoma, analyzes clinical and histological features and it was classifies as a separate entity. The term "odonto COC" was suggested. The anterior region of the jawbones was the most commonly affected site in both cases, but the COC with odontome presents a female predominance (2:1) with a mean age of 16 years, most frequently occurring in the maxilla (61.5%). Radiographically appears as a mixed radiolucent-radiopaque lesion (80.5%). Microscopically the epithelial components in COC with odontome were identical to those described for simple COC, but the former present's tooth-like structures that appear to be an integral part of the lesion.

Several possibilities are suggested regarding the pathogenesis of COC with odontome. One possibility is that the COC and the odontoma may represent coincidental juxtaposition of COC and an odontoma, because other odontogenic tumors like ameloblastoma have been reported to be associated with COC. [5] Other investigators suggest that the COC develops secondarily from odontogenic epithelium that participates in the formation of the odontoma. However, it also has been suggested that the odontoma develops secondarily from lining epithelium of the COC. [6]

The etiology of odontoma is not known but environmental causes such as infection, trauma, family history and genetic mutation are hypothesized. [7],[8] The complex odontoma constitute 5-30% of all odontogenic tumors and are mostly found in the posterior mandible and anterior maxilla. They are seen with unerupted teeth in 10-44% and about 17% of them are associated with impacted maxillary lateral incisors. Females are affected marginally more than male (1.5:1). World Health Organization classified odontomes into three groups: (a) complex odontome; when the calcified dental tissues are simply arranged in an irregular mass bearing no morphological similarity to rudimentary teeth. (b) Compound odontoma: composed of all odontogenic tissues in an orderly pattern, which result in many teeth-like structures, but without morphological resemblance to normal teeth. (c) Ameloblastic fibro-odontome: consists of varying amounts of calcified dental tissue and dental papilla-like tissue, the later component resembling an ameloblastic fibroma. [8]

The ameloblastic fibro-odontome is considered as an immature precursor of complex odontome. Odontomes are incidental findings on routine radiographs. Retention of deciduous teeth, unerupted permanent teeth, cortical expansion and teeth displacement are all indicators for a possible odontomes. [9] They are symptomatic with expansion, local and/or radiating pain and neurosensory deficit when secondarily infected. [10] The differential diagnosis of complex odontoma include cementifying or ossifying fibroma, adenomatoid odontogenic tumor, COCs, periapical cemental dysplasia and calcifying epithelial odontogenic tumor. [11]


  Conclusion Top


COC occurring along with complex odontoma is a rare finding among the cystic lesions of the jaws. Early diagnosis and management is important to prevent its potential complications like involvement of the nasal floor and maxillary sinus and progression to more aggressive tumor masses thus causing severe morbidity.

 
  References Top

1.Gorlin RJ, Pindborg JJ, Odont, Clausen FP, Vickers RA. The calcifying odontogenic cyst - A possible analogue of the cutaneous calcifying epithelioma of Malherbe. An analysis of fifteen cases. Oral Surg Oral Med Oral Pathol 1962;15:1235-43.  Back to cited text no. 1
    
2.Freedman PD, Lumerman H, Gee JK. Calcifying odontogenic cyst. A review and analysis of seventy cases. Oral Surg Oral Med Oral Pathol 1975;40:93-106.  Back to cited text no. 2
    
3.Praetorius FP. Calcifying odontogenic cyst. Range, variation and neoplastic potential. Symposium on maxillofacial bone pathology. Int J Oral Surg 1975;4:89.  Back to cited text no. 3
    
4.Hirshberg A, Kaplan I, Buchner A. Calcifying odontogenic cyst associated with odontoma: A possible separate entity (odontocalcifying odontogenic cyst). J Oral Maxillofac Surg 1994;52:555-8.  Back to cited text no. 4
    
5.Lello GE, Makek M. Calcifying odontogenic cyst. Int J Oral Maxillofac Surg 1986;15:637-44.  Back to cited text no. 5
    
6.Shamaskin RG, Svirsky JA, Kaugars GE. Intraosseous and extraosseous calcifying odontogenic cyst (Gorlin cyst). J Oral Maxillofac Surg 1989;47:562-5.  Back to cited text no. 6
    
7.Toida M, Ishimaru J, Tatematsu N. Calcifying odontogenic cyst associated with compound odontoma: Report of a case. J Oral Maxillofac Surg 1990;48:77-81.  Back to cited text no. 7
    
8.Philipsen HP, Reichart PA, Praetorius F. Mixed odontogenic tumours and odontomas. Considerations on interrelationship. Review of the literature and presentation of 134 new cases of odontomas. Oral Oncol 1997;33:86-99.  Back to cited text no. 8
    
9.Phillips MD, Closmann JJ, Baus MR, Torske KR, Williams SB. Hybrid odontogenic tumor with features of ameloblastic fibro-odontoma, calcifying odontogenic cyst, and adenomatoid odontogenic tumor: A case report and review of the literature. J Oral Maxillofac Surg 2010;68:470-4.  Back to cited text no. 9
    
10.Oliveira JA, da Silva CJ, Costa IM, Loyola AM. Calcifying odontogenic cyst in infancy: Report of case associated with compound odontoma. ASDC J Dent Child 1995;62:70-3.  Back to cited text no. 10
    
11.Ozeç I, Kiliç E, Yeler H, Göze F, Yeler D. Large complex odontoma associated with a primary tooth. Quintessence Int 2007;38:521-4.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]



 

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