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| CASE REPORT |
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| Year : 2020 | Volume
: 12
| Issue : 2 | Page : 91-97 |
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Proliferating ameloblastomatous calcifying odontogenic cyst: A rare case report
Monika Negi1, Abhiney Puri1, Rajat Nangia1, Namita Sepolia2
1 Department of Oral Pathology Microbiology and Forensic Odontology, HIDS, Paonta Sahib, Himachal Pradesh, India
2 Department of Oral Pathology Microbiology and Forensic Odontology, Bhojia Dental Collge and Hospital, Baddi, Himachal Pradesh, India
| Date of Submission | 12-Jun-2019 |
| Date of Acceptance | 31-Dec-2019 |
| Date of Web Publication | 22-Jul-2020 |
Correspondence Address:
Monika Negi
Department of Oral Pathology Microbiology and Forensic Odontology, HIDS, Paonta Sahib, Himachal Pradesh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jorr.jorr_22_19
Calcifying odontogenic cyst (COC) has many traits of an odontogenic tumor and has in veracity been categorized as such in the World Health Organization publication histologic typing of odontogenic tumors. COC is an unconventional developmental odontogenic cyst first recited by Gorlin in 1962. It is a rare pathology and accounts for only 1%–2% of jaw cyst reported. There has always been a question whether its a cyst, neoplasm, or hematoma. Ameloblastoma is one of the well-known odontogenic tumors that can be conglomerated with COC but only a few reports include subjective clinical and radiographic features. Here, we present a specified case report of the COC with ameloblastic proliferation which is an exceptionally rare histologic variant. The classical histological features of lining epithelium and characteristic ghost cells are seen sideways with ameloblastomatous proliferations. The case is ventilated here because of its singularity.
Keywords: Ameloblastoma, calcification, calcifying odontogenic cyst, cyst, odontogenic, tumor
How to cite this article:
Negi M, Puri A, Nangia R, Sepolia N. Proliferating ameloblastomatous calcifying odontogenic cyst: A rare case report. J Oral Res Rev 2020;12:91-7 |
How to cite this URL:
Negi M, Puri A, Nangia R, Sepolia N. Proliferating ameloblastomatous calcifying odontogenic cyst: A rare case report. J Oral Res Rev [serial online] 2020 [cited 2023 Mar 27];12:91-7. Available from: https://www.jorr.org/text.asp?2020/12/2/91/290502 |
| Introduction | |  |
The cyst itself is classified as cyst of odontogenic epithelial origin which was marked by its histological resemblance to cutaneous calcifying epithelioma of Shear.[1] The calcifying odontogenic cyst (COC) is an atypical lesion first described by Gorlin et al. in 1962.[2] In 1971, World Health Organization (WHO) classified COC as a cystic lesion.[3] The ameloblastomatous proliferative COC is cataloged as Type III in cystic variety. The WHO in 2005 had classified COC in the group of odontogenic tumors having odontogenic epithelium with odontogenic ectomesenchyme with/without dental hard tissue formation which now in 2017 has been reclassified in the group of odontogenic cysts. It is well known that the lesion is often associated with the tumors of odontogenic origin such as odontoma, ameloblastoma, and adenomatoid odontogenic tumor.
It is an uncommon lesion and accounts from 1% to 2% of all odontogenic jaw cysts.[4] Histopathologic features is characterized by the presence of a variable number of epithelial cells without nuclei called ghost cells, which may be calcified.[5]
It is well known that this lesion recurrently occurs in association with odontogenic tumors such as complex odontoma and ameloblastoma, and all contemporary histopathological classification of COCs advocate classifying the variants concurrent with these tumors.[6] Although the association of ameloblastoma with this lesion is important, there are not many reports that depicts the clinical and radiographic features of COC correlated with ameloblastoma. In this manuscript, we account a case of ameloblastomatous COC that occurred in the mandible of a 25-year-old male. Ameloblastomatous COC resembles unicystic ameloblastoma except for ghost cells and calcifications within the proliferative epithelium and the fact that it occurs only intraosseously. Newer guidelines by the WHO have mentioned it in the group of cysts.
Various terminologies were given by different authors are tabulated in the [Table 1] and classifications in [Table 2] and [Table 3]. | Table 2: Classification of calcifying odontogenic cyst proposed by Praetorius et al.
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 | Table 3: Classification of calcifying odontogenic cyst proposed by Buchner
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[4],[7],[6] The purpose of this article is to present a case of ameloblastomatous COC along with a systemic review in the scientific literature.
| Case Report | | |
A 25-year-old Indian male was referred to the department of OMDR, HIDS complaining of swelling on the right side of the lower jaw for 2 months [Figure 1], [Figure 2], [Figure 3]. On clinical examination, overlying skin appeared normal with the local rise in temperature. On intraoral examination, a swelling extending from the premolar to ramus of the mandible with the buccolingual expansion of cortical bone is seen. Hard tissue examination did not reveal decay or mobility of teeth. Pulp vitality testing showed no response in the region. | Figure 3: Clinical intraoral photograph showing swelling with intact overlying mucosa
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Radiographic findings
OPG revealed a well-defined multilocular radiolucency from the right lower second molar to the right ramus with a remarkable bony expansion toward buccal and lingual sides. An extreme radiolucency involving complete ramus with few radiopaque septa was noticed with respect to impacted 48. [Figure 4]. | Figure 4: Orthopantomograph demonstrating a unilocular radiolucency involving complete ramus of the mandible associated with radiopacity with impacted tooth 48
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Clinical diagnosis of the dentigerous cyst was made. An incisional biopsy from the right retromolar area was performed to establish a definitive diagnosis. During the biopsy, a cystic lesion filled with the clear pale fluid was seen.
Histological features
Under scanner view, thin cystic lining overlying fibro cellular connective tissue wall was seen [Figure 5]. Under lower magnification cystic lining with prominent basal cell layer with the palisading arrangement was seen. Cyst lining lined with basal cell layer resembling ameloblast cells was also evident. Cells above the basal cell layer were seen loosely arranged resembling stellate reticulum. Numerous ameloblast islands along with Ghost cells with distinct cell outline and numerous spherical calcifications were seen along with epithelium and connective tissue wall. Juxtaepithelial hyalinization was also seen. Connective tissue seen was fibrocellular in nature with chronic inflammatory cells, calcifications, and Ghost cells. Few endothelial cell-lined blood vessels with engorged RBCs were also seen [Figure 6], [Figure 7], [Figure 8], [Figure 9]. Histopathological features seen under lower magnification were confirmed under highr magnification [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14]. | Figure 5: Photomicrograph showing proliferating cystic lining (H and E, ×4)
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 | Figure 6: Photomicrograph showing proliferating cystic lining with stellate reticulum like areas and spherical to irregular calcifications and juxtaepithelial hyalinization (H and E, ×10)
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 | Figure 7: Photomicrograph showing ameloblastic cells with stellate reticulum along with numerous ghost cells (H and E, ×10)
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 | Figure 8: Photomicrograph showing dentinoid and ghost cells (H and E, ×10)
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 | Figure 9: Photomicrograph of cyst lining showing ghost cells and calcifications (H and E, ×10)
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 | Figure 10: Photomicrograph showing acanthomatous ameloblastomatous ameloblastic proliferations in the connective tissue. Basal cell hyperchromatism, vacuolization, and nuclear polarization (×40)
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 | Figure 11: Photomicrograph showing ameloblastic cells with stellate reticulum along with numerous ghost cells (H and E, ×40)
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 | Figure 13: Cystic capsule lined with epithelium composed of a well-defined basal layer of cuboidal cells with reversal of polarity and juxtaepithelial hyalinisation
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 | Figure 14: Odontogenic epithelial islands seen adjacent to the calcified mass showing evidence of ghost cell formation
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These features were confirmatory for ameloblastomatous COC.
| Discussion | | |
The COC is an exceptionally rare entity, with the ameloblastomatous type being even rare. Since the first depiction by Gorlin et al.,[2] many cases of COC have been reported in the literature; but, in the mainstream, only a few cases have been reported at a time. This cyst is not common in the gnathic bones, representing about less than 6% of all odontogenic lesions.[8] Many authors have made efforts to clarify clinical and radiographic features of COC by analyzing a large number of reported cases, and now, the following extensive features may be accepted. A pronounced peak frequency of COC in the second decade has been reported.[6],[7],[8],[9] In 1971, WHO defined COC as “a nonneoplastic cystic lesion, in which the epithelial lining shows a well-defined basal layer of columnar cells, an overlying layer that is often many cell layers thick that may resemble stellate reticulum and masses of ghost cells that may be in the epithelial cyst lining or in the fibrous capsule. The ghost cells may become calcified. Next, to the basal layer of the epithelium, dysplastic dentin may be laid down.”[10]
COC occurs intraosseously or extraosseously with intraosseous being prominent. Before estrangement of this entity by Gorlin et al., it was repeatedly considered as specific form of ameloblastoma.[2] The COC is an exceptional lesion demonstrating the considerable histologic diversity and presenting with variable clinical behavior. It is broadly considered to exemplify a cyst; some investigators prefer to classify it as a neoplasm.[3] On reviewing the literature, we found the reporting of twenty-one cases of which Hong et al. reviewed ninety-two cases of COCs which were divided into 85.9% cases of cysts and 14.1% cases of neoplasms. From these ninety-two cases, eleven cases were of ameloblastomatous COC which were characterized by ameloblastoma-like, cyst-lining epithelium with ghost cells and calcifications. Nine of eleven cases occurred in mandible and two in maxilla. In their article, they did not give details such as bone resorption, radiolucency, or recurrence rate. Thus, we have not included their research in our review.
Query concerning the nature of cyst emerged to be classified by Toida, who recently categorize COC into a cyst or neoplasm. In the new classification of WHO (2005), the term calcifying cystic odontogenic tumor was replaced by COC which constitutes a benign cystic neoplasia presenting an epithelium with ghost cells which may display calcification in it.[11]
According to Praetorius et al., the cystic lesion can be divided into three basic types:
- Simple unicystic
- Unicystic odontoma producing
- Unicystic ameloblastomatous producing type.[7]
Microscopically, ameloblastomatous COC resembles unicystic ameloblastoma except for the ghost cells and calcifications within the proliferative epithelium. Ameloblastomatous COC occurs only intraosseously. Individual ghost cells may fuse together to form large sheets of amorphous eosinophilic structures on which calcification may occur. Irregular masses of calcified structures suggestive of dysplastic dentin are present in association with basal layer.[12] Radiographically, COC generally appears as the unilocular lesion with a well-defined margin. The presence of calcifications which are observed in about half of them,[6],[13] is the most important radiographic feature for the diagnosis of the COC.
This subtype of COC needs to be differentiated from the ameloblastoma arising in COC as the former requires conservative form of management while the latter demands for aggressive measures for its treatment. In contrast to ameloblastoma, In COC dystrophic calcifications and ghost cells are seen within the proliferative epithelium. Also, Vickers and Gorlin criteria is not seen. Ameloblastoma ex COC designates an ameloblastoma arising from the cyst-lining epithelium of COC.[14]
From the genetic point of view, missense mutations in CTNNB1 which encodes beta-catenin were found in 91% of the cases studied by Yukimori et al.,[15] and this activation of beta-catenin abolishes the phosphorylation sites Asp32, Ser33, or Ser37. Mutations in CTNNB1 are the major driver mutations of CCOT, and that CCOT is a genetic analog of pilomatrixoma and adamantinomatous craniopharyngioma in the odontogenic tissue.
Tajima et al.[16] in their article presented a case of a 35-year-old male patient with well-demarcated cystic lesion in mandibular symphysis region. Radiographically, radiolucency extended between mandibular premolar regions bilaterally with root resorption of the anterior teeth. Lesion was surgically removed, and histopathologic report gave ameloblastoma ex COC as the final diagnosis. There was no recurrence after a follow-up of 5 years. Aithal et al.[13] reported a case in a 28-year-old female with a hard, nontender swelling in the left posterior region of the mandible. Radiograph showed a multilocular radiolucency from canine to second molar. Microscopic examination revealed ghost cells in cystic epithelium and juxtaepithelial hyalinization. Odontogenic epithelium formed rosettes, and acanthomatous ameloblastic islands were also seen in the connective tissue lining of the cyst. It was surgically excised and there was no recurrence at 2 years follow-up. Final diagnosis of ameloblastomatous COC was made.
Iida et al.[17] presented a case in a 17-year-old male with a tender bony swelling in the right mandibular body. Radiographs revealed a well-defined multilocular lesion in the right side of mandible in the ramus region with buccolingual expansion with displacement of lower second molar below the first molar. The tumor was treated with a more conservative approach of enucleation followed by ostectomy with no recurrence even after 13 years. Final diagnosis of ameloblastomatous COC was given. Iida et al.[18] again concluded that the impaction and displacement of teeth in cases affecting the posterior jaw segment can be indicative of the development of COC. The presence of impaction or displacement of teeth for COCs occurring in the posterior segments of the jaw and absence of this finding for anteriorly placed lesions may be indicative of the timing of COC development.
Ledesma-Montes et al.[11] reviewed the clinicopathological features of 122 CCOT, DGCT, and GCOC cases from 14 institutions in seven countries of three different continents and concluded that of all the cases, ameloblastomatous proliferating type of CCOT accounted for only three cases (2.46%) indicating the lesion to be very rare. They described the lesion having intraluminal or capsular plexiform growths similar to those seen in the plexiform variant of cystic Ameloblastoma.
In our case, wide surgical excision of the lesion with normal margins followed by chemical cauterization of Carnoy's solution was done. There has been no recurrence during these 4 years of follow-up period.
Among these, ameloblastomas may be most important in terms of its histopathology and management. So far, very few cases have been documented on ameloblastomatous COC. However, there have been a considerable number of reports of ameloblastoma arising from a variety of odontogenic cysts.[19] Therefore, it is not surprising that the cyst-lining epithelium of COC can be transformed into a true ameloblastoma that may fulfill the criteria of Vickers and Gorlin.[14] Whether to have a conservative or a radical approach for management of this lesion has been an issue of debate. Several authors have suggested that if COC is associated with ameloblastoma, its behavior and progression is not of COC and hence should be treated more aggressively. Our case did not show any recurrence after its thorough excision with normal margins.
| Conclusion | | |
COC with the ameloblastomatous exposition is an erratic histologic variant and there are not many recounted cases of a COC with ameloblastomatous proliferation; so, more number of cases should be brought to literature to understand its biological comportment.
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 | | |
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| 14. | Vickers RA, Gorlin RJ. Ameloblastoma: Delineation of early histopathologic features of neoplasia. Cancer 1970;26:699-710. |
| 15. | Yukimori A, Oikawa Y, Morita KI, Nguyen CTK, Harada H, Yamaguchi S, et al. Genetic basis of calcifying cystic odontogenic tumors. PLoS One 2017;12:e0180224. |
| 16. | Tajima Y, Yokose S, Sakamoto E, Yamamoto Y, Utsumi N. Ameloblastoma arising in calcifying odontogenic cyst. Report of a case. Oral Surg Oral Med Oral Pathol 1992;74:776-9. |
| 17. | 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. |
| 18. | Iida S, Fukuda Y, Ueda T, Aikawa T, Arizpe JE, Okura M. Calcifying odontogenic cyst: Radiologic findings in 11 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:356-62. |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14]
[Table 1], [Table 2], [Table 3]
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