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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 28  |  Issue : 4  |  Page : 428-432

Calcifying cystic odontogenic tumor associated with unicystic ameloblastoma: Report of a rare hybrid odontogenic tumor


1 Department of Oral Medicine and Radiology, Dr. Syamala Reddy Dental College, Hospital and Research Centre, Bengaluru, Karnataka, India
2 Department of Oral and Maxillofacial Surgery, Dr. Syamala Reddy Dental College, Hospital and Research Centre, Bengaluru, Karnataka, India

Date of Submission22-Mar-2016
Date of Acceptance23-Dec-2016
Date of Web Publication21-Feb-2017

Correspondence Address:
Dr. Laboni Ghorai
Room No. 1, Dr. Syamala Reddy Dental College, Hospital and Research Centre, #111/1, SGR College Main Road, Munnekolala, Marathahalli (Post), Bengaluru - 560 037, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.200643

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   Abstract 

A hybrid odontogenic tumor comprising two distinct lesions is extremely rare. In this paper, we report one such case of hybrid odontogenic tumor composed of calcifying cystic odontogenic tumor and unicystic ameloblastoma in the left maxillary anterior region of a 30-year-old Indian male. Calcifying cystic odontogenic tumor (CCOT) is a benign cystic neoplasm of odontogenic origin, which involves association of a cyst (calcifying odontogenic cyst) with an odontogenic tumor, a finding which is a rare event in other types of odontogenic cysts or tumors. CCOT associated with unicystic ameloblastoma is a rare entity with very few cases being documented in literature. Preoperative diagnosis of such hybrid lesions can be difficult or sometimes impossible because it shares significant clinical and radiographic similarities with odontogenic cysts and tumors.

Keywords: Calcifying cystic odontogenic tumor, calcifying odontogenic cyst, unicystic ameloblastoma


How to cite this article:
Asha ML, Ghorai L, Lokesha U, Kumar HM. Calcifying cystic odontogenic tumor associated with unicystic ameloblastoma: Report of a rare hybrid odontogenic tumor. J Indian Acad Oral Med Radiol 2016;28:428-32

How to cite this URL:
Asha ML, Ghorai L, Lokesha U, Kumar HM. Calcifying cystic odontogenic tumor associated with unicystic ameloblastoma: Report of a rare hybrid odontogenic tumor. J Indian Acad Oral Med Radiol [serial online] 2016 [cited 2021 Jan 18];28:428-32. Available from: https://www.jiaomr.in/text.asp?2016/28/4/428/200643


   Introduction Top


Calcifying odontogenic cyst (COC) is an uncommon developmental odontogenic cyst first categorized as a distinct entity by Gorlin et al. in 1962.[1] It represents approximately 1% of all cysts of the jaws.[2] Previous reports have revealed the coexistence of COC with other odontogenic tumors. The most common of these is odontoma, but rarely, ameloblastoma, adenomatoid odontogenic tumor, odontoameloblastoma, ameloblastic fibroma, ameloblastic fibro-odontoma and odontogenic myxofibroma have been identified.[3] Hence, the World Health Organization (WHO) in 2005 reclassified it as calcifying cystic odontogenic tumor (CCOT).[4] Herein, we report a rare case of CCOT associated with unicystic ameloblastoma.


   Case Report Top


A 30-year-old male patient reported with the chief complaint of a painless swelling on the left side of the face since 1 year. The swelling had gradually increased in size to the present dimension. Patient gave a past history of trauma to the face due to a road traffic accident 1 year back. His past medical, surgical and dental histories were noncontributory. General physical examination revealed no significant abnormality.

Extraoral examination revealed a solitary diffuse swelling, roughly oval in shape and measuring approximately 4 × 5 cm, in the left middle one-third of face, extending superoinferiorly from 2 cm below the infraorbital rim up to an imaginary line joining the left corner of the mouth and tragus of the left ear and anteroposteriorly from 2 cm to the right of philtrum crossing the midline and extending till 5 cm anterior to the tragus, causing obliteration of the left nasolabial fold and elevation of left ala of nose [Figure 1]. On palpation, swelling was nontender and bony-hard in consistency. Intraoral examination revealed obliteration of the upper left labial vestibule from mesial aspect of 12 to distal aspect of 24 with no evidence of sinus opening, pus discharge or bleeding [Figure 2]. On palpation, swelling was nontender, bony hard posteriorly, soft and fluctuant anteriorly. Nontender, bony-hard extension of the swelling was noted in the left anterior hard palate [Figure 3]. Hard tissue examination revealed discolored 21, displaced 22 and 23 and mobility of 21, 22 and 23. Vitality test with respect to the related teeth revealed nonvital 21 and 22. Fine needle aspiration through the area of fluctuance yielded straw-colored fluid, and the protein content of the fluid was estimated to be 7.1 mg/dl. Based on the patient's history, clinical findings and chairside investigations, a provisional diagnosis of radicular cyst in relation to 21 and 22 was made.
Figure 1: Extraoral swelling on the left side of the face

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Figure 2: Intraoral swelling showing labial cortical expansion

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Figure 3: Palatal extension of the intraoral swelling

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Further, the patient was subjected to radiographic examinations. Intraoral periapical radiograph revealed obliteration of the pulp chamber and root canal of 22, external root resorption in relation to 21 and 22, and an alteration in trabecular pattern [Figure 4]. Maxillary anterior occlusal radiograph revealed a well-defined radiolucency with localized honeycomb-like multilocular pattern involving the left anterior dentoalveolar region along with expansion and thinning of the left labial cortex [Figure 5]. Similar pattern of the lesion was also noted in panoramic radiograph, where the lesion extended anteroposteriorly from the mesial aspect of 12 to periapical area of 24 and superoinferiorly from the floor of the nasal fossa to the crestal bone, with the epicentre of the lesion being in the interdental area of 22 and 23 [Figure 6]. To know the clear extension of the lesion, cone-beam computed tomography (CBCT) of the anterior maxilla was performed, which revealed a well-defined predominantly hypodense lesion with localized hyperdense areas. The three-dimensional image reconstruction provided better visualization of palatal perforation, expansion, thinning and perforation of labial cortex and displacement and thinning of left nasal fossa floor [Figure 7] and [Figure 8]. Based on these findings, radiographic diagnosis of benign nonaggressive type of odontogenic lesion was made, and CCOT, adenomatoid odontogenic tumor, central giant cell granuloma, and ameloblastoma and its variants were considered in the differential diagnosis.
Figure 4: Intraoral periapical radiograph showing obliteration of pulp chamber and root canal of 22, root resorption of 21 and 22, and an alteration in trabecular pattern

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Figure 5: Maxillary anterior occlusal radiograph showing a well-defined radiolucent lesion with localized honeycomb-like multilocular pattern causing expansion and thinning of the left labial cortex

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Figure 6: Panoramic radiograph showing extent of lesion from the mesial aspect of 12 to the periapical area of 24

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Figure 7: CBCT images showing a well-defined predominantly hypodense lesion with localized hyperdense areas in axial, coronal, and sagittal sections

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Figure 8: Three-dimensional volume rendered image (arrows showing palatal perforation, expansion, thinning, and perforation of labial cortex and displacement and thinning of left nasal fossa floor)

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Incisional biopsy was performed under local anesthesia. The histologic analysis of the specimen revealed proliferation of odontogenic epithelial islands with organization into ameloblastomatous pattern and the connective tissue resembled odontogenic mesenchyme with hard tissue formation suggestive of mixed odontogenic lesion of very low aggressive potential. The patient was treated surgically under general anesthesia. Enucleation and curettage of the lesion followed by application of Carnoy's solution and extraction of teeth closely related to the lesion was done. Histopathological examination of the excised lining revealed proliferation of odontogenic epithelium into ameloblastomatous pattern, presence of ghost cells in focal areas, dentinoid type of hard tissue formation in connective tissue and juxtaepithelial hyalinization [Figure 9]. Based on these features, a histopathological diagnosis of CCOT associated with unicystic ameloblastoma was confirmed. The patient is on regular follow-up [Figure 10] and has reported a healthy and disease-free life since the surgery.
Figure 9: Photomicrograph showing proliferation of odontogenic epithelium into ameloblastomatous pattern (red arrows), ghost cells in focal areas (blue arrows), hard tissue formation in connective tissue (black arrow), and juxtaepithelial hyalinization (green arrow)

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Figure 10: 1-month postoperative photograph of the patient showing correction of extraoral deformity and intraoral soft tissue healing

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   Discussion Top


CCOT is an uncommon lesion exhibiting variable clinical behavior and considerable histologic diversity. It is widely considered to represent a cyst, although some investigators prefer to classify it as a neoplasm.[5] Association of CCOT with unicystic ameloblastoma is rare, and hence, determination of the most common age, sex and location of this lesion is difficult. According to the case reports in literature, as presented in [Table 1], most patients are between 10 and 30 years of age, no sex predilection has been noted and the tumor tends to involve the posterior region of the mandible.[6],[7],[8],[9],[10],[11] In the present case, the age of occurrence is consistent with that described in literature and clinical presentation, as an asymptomatic facial swelling, is typical for such lesion. However, the site of occurrence in anterior maxilla is rare and has not been reported in English literature.
Table 1: Cases of CCOT associated with unicystic ameloblastoma

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Radiographically, CCOTs generally appear as well-defined unilocular lesions with presence of calcifications in approximately half of the cases. Root resorption or root divergence may also be observed.[7],[12] However, in the present case, the radiographs revealed well-defined corticated, predominantly radiolucent lesion with localized multilocular pattern. CBCT can be helpful in establishing the extent of the lesion, particularly when located in the maxilla.

Histopathologically, Hong et al. advocated two categories of CCOT associated with ameloblastoma, namely, ameloblatomatous cystic variant and neoplastic variant associated with ameloblastoma. The former is characterized by unicystic structure with the lining epithelium showing unifocal or multifocal ameloblastomatous proliferation along with isolated or clustered ghost cells and calcifications. Histopathologically, the latter is characterized by few or no ghost cells with calcification in the transformed ameloblastomatous epithelial portion, while the cystic epithelial lining contains considerable number of ghost cells and calcifications. In the present case, presence of ghost cells in the ameloblastomatous epithelial proliferations is consistent with the features of ameloblastomatous CCOT.[6]

Regarding management, Buchner suggested that, if the CCOT was associated with an ameloblastoma, its behavior and prognosis would be that of an ameloblastoma, not that of CCOT.[12] Hence, in this case, treatment of unicystic ameloblastoma has been advocated. Among the various conservative management modalities that have been tried, application of Carnoy's solution following enucleation and curettage of the cystic lesions seems to be a promising solution and has resulted in a recurrence rate of 16%, which is the best except for resection. The recurrence rate could even be lower than reported if the closely-related teeth with tumor are rejected.[13] In the present case, considering the age of the patient, anterior location and well-corticated boundary of the lesion and minimal involvement of the vital structures, similar conservative treatment has been undertaken with no recurrence being reported till date.

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.
Mashhadi Abbas F, Moshref M, Sargolzaie S, Kargahi N. Ameloblastomatous calcifying cystic odontogenic tumor: A rare histologic variant. Shiraz Univ Dent J 2009;9:45-8.  Back to cited text no. 1
    
2.
Gorlin RJ, Pindborg JJ, 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. 2
    
3.
Yoon JH, Kim HJ, Yook JI, Cha IH, Ellis GL, Kim J. Hybrid odontogenic tumour of calcifying odontogenic cyst and ameloblastic fibroma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98:80-4.  Back to cited text no. 3
    
4.
Barnes L, Eveson JW, Reichart P, Sidransky D. World Health Organization Classification of Tumours. Pathology and genetics of head and neck tumours. Lyon: IARC Press; 2005. p. 313.  Back to cited text no. 4
    
5.
Nosrati K, Seyedmajidi M. Ameloblastomatous calcifying odontogenic cyst: A case report of a rare histologic variant. Arch Iran Med 2009;12:417-20.  Back to cited text no. 5
    
6.
Hong SP, Ellis GL, Hartman KS. Calcifying odontogenic cyst. A review of ninety two cases with re-evaluation of their nature as cysts or neoplasm, the nature of ghost cells, and subclassification. Oral Surg Oral Med Oral Pathol 1991;72:56-64.  Back to cited text no. 6
    
7.
Aithal D, Reddy BS, Mahajan S, Boaz K, Kamboj M. Ameloblastomatous calcifying odontogenic cyst: A rare histologic variant. J Oral Pathol Med 2003;32:376-8.  Back to cited text no. 7
    
8.
Iida S, Ueda T, Aikawa T, Kishino M, Okura M, Kogo M. Ameloblastomatous calcifying odontogenic cyst in the mandible. Dentomaxillofac Radiol 2004;33:409-12.  Back to cited text no. 8
    
9.
Kamran N, Maryam S. Ameloblastomatous calcifying odontogenic cyst: A case report of a rare histologic variant. Arch Iranian Med 2009;12:417-20. Buchner A. The central (intraosseous) calcifying odontogenic cyst: An analysis of 215 cases. J Oral Maxillofac Surg 1991;49:330-9.  Back to cited text no. 9
    
10.
Kallalli BN, Rawson K, Patel V, Patil S. Ameloblastomatous calcifying odontogenic cyst: A rare histologic variant. J Indian Acad Oral Med Radiol 2015;27:123-6.  Back to cited text no. 10
  Medknow Journal  
11.
Khot DK, Warke DD, Manjrekar DKB, Madne DS. Calcifying cystic odontogenic tumor associated with Unicystic Ameloblastoma: A unique entity– A Case report. SEAJCRR 2015;4:1270-80.  Back to cited text no. 11
    
12.
Buchner A. The central (intraosseous) calcifying odontogenic cyst: An analysis of 215 cases. J Oral Maxillofac Surg 1991;49:330-9.  Back to cited text no. 12
    
13.
Lau SL, Samman N. Recurrence related to treatment modalities of unicystic ameloblastoma: A systematic review. Int J Oral Maxillofac Surg 2006;35:681-90.  Back to cited text no. 13
    


    Figures

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

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