Ameloblastomatous calcifying cystic odontogenic tumor: A review of a rare lesion

Aastha Manchanda; Seema Patil

doi:10.4103/jiaomr.jiaomr_181_19

REVIEW ARTICLE

Year : 2019 | Volume : 31 | Issue : 4 | Page : 370-373

Ameloblastomatous calcifying cystic odontogenic tumor: A review of a rare lesion

Aastha Manchanda¹, Seema Patil²
¹ Department of Oral Medicine and Radiology, Inderprastha Dental College and Hospital, Sahibabad, Ghaziabad, Uttar Pradesh, India
² Department of Oral Medicine and Radiology, D.A. Pandu Memorial R.V. Dental College, Bengaluru, Karnataka, India

Date of Submission	02-Nov-2019
Date of Acceptance	04-Feb-2020
Date of Web Publication	03-Mar-2020

Correspondence Address:
Dr. Aastha Manchanda
Department of Oral Medicine and Radiology, Inderprastha Dental College and Hospital, 46/1, Site IV Industrial Area, Sahibabad, Ghaziabad - 201 010, Uttar Pradesh
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jiaomr.jiaomr_181_19

Abstract

Calcifying cystic odontogenic tumor (CCOT) is an uncommon developmental lesion accounting for less than 2% of odontogenic tumors. Because of its diverse histopathology, there has always been confusion about its nature as a cyst or a neoplasm, although it is well known that this lesion often occurs in association with other odontogenic tumors such as complex odontoma and ameloblastoma. It is noted that if CCOT is associated with an ameloblastoma, its behavior and prognosis will be that of an ameloblastoma, not of a CCOT. This article presents a review of all reported cases of ameloblastomatous CCOT in the last 15 years in journals indexed with PubMed.

Keywords: Ameloblastomatous CCOT, ameloblastoma in CCOT, calcifying cystic odontogenic tumor (CCOT)

How to cite this article:
Manchanda A, Patil S. Ameloblastomatous calcifying cystic odontogenic tumor: A review of a rare lesion. J Indian Acad Oral Med Radiol 2019;31:370-3

How to cite this URL:
Manchanda A, Patil S. Ameloblastomatous calcifying cystic odontogenic tumor: A review of a rare lesion. J Indian Acad Oral Med Radiol [serial online] 2019 [cited 2022 May 26];31:370-3. Available from: https://www.jiaomr.in/text.asp?2019/31/4/370/279861

Introduction

Calcifying cystic odontogenic tomor (CCOT) is a rare entity accounting for less than 2% of all odontogenic tumors.^[1],[2],[3] It was originally described by Gorlin, Pindborg, Praetorius-Clausen, and Vickers in 1962 as Calcifying odontogenic cyst (COC).^[4] The lesion since its discovery has been found to exhibit cystic, benign tumor like and rare malignant characteristics.^[5] A wide range of histopathological appearances were also noted.

Thus, its diverse clinical behavior and histopathologic characteristics prompted the usage of several terminologies and classifications. Gold in 1963 named the lesion as “Keratinizing calcifying odontogenic cyst.” Fejerskov and Krogh in 1972 called it “Calcifying ghost cell odontogenic tumor,” whereas Freedman et al. in 1975 suggested the name “Calcifying cystic odontogenic tumor (CCOT).”^[5]

Praetorius et al. in 1981 divided the lesion into two groups, 1) cystic and 2) neoplastic, considering the different histologic patterns in them. The cystic form was further divided into (1a) simple unicystic, (1b) odontoma producing, and (1c) ameloblastomatous proliferating. The neoplastic counterpart was named “Dentinogenic ghost cell tumor (DGCT).”^[6]

Subsequently, WHO in 2005 renamed COC as CCOT and the term DGCT was retained. A third malignant counterpart was found and the name “Ghost cell odontogenic carcinoma (GCOC)” was proposed for the same. This classification has been widely accepted and is currently in use.^[6]

Among the several forms of CCOT, the simple unicystic form of CCOT is most commonly encountered followed by the odontome associated CCOT.^[6] Although the association of ameloblastoma with this lesion is important, there are only a few reports that detail the clinical and radiographic features of CCOT associated with ameloblastoma.

In this article, we present an extensive review about the clinical and radiographic features of case reports of ameloblastomatous CCOT published in PubMed research base in the last 15 years (2003–2018). PubMed was extensively searched for all reported cases of ameloblastomatous COC using keywords such as ameloblastomatous COC, amelobolastomatous CCOT, and gorlin cyst with ameloblastic proliferation [Table 1].

Table 1: AMELOBLASTOMATOUS CCOT

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Discussion

CCOT is thought to originate from the remnants of odontogenic epithelium entrapped within the maxilla/mandible or gingival tissue giving rise to intraosseous or extraosseous forms, respectively.^[7] The intraosseous form is more common with a prevalence rate of 70%.^[8]

In general, CCOT has been found to occur over a wide age range of 1 to 82 years,^{[2],[18],[19]} and bimodal peak of occurrence of the lesion in the 2^nd and 7^th decade has also been reported.^[18],[20] This probably correlates with the age of occurrence of the intraosseous and extraosseous variants, respectively.^[2] Similar to simple CCOT, most case reports of the ameloblastomatous type mentioned above revealed a predilection in the younger age group, that is, in 2^nd or 3^rd decades of lives although cases have been reported in 5 years^[16] and 64 years^[15] as well. According to the abovementioned reports, no definite gender predilection could be expected for this lesion.

CCOT lesion is equally distributed between the maxilla and mandible,^[17],[19] although case reports suggest a preponderance in the maxilla,^[10] especially for the simple unicystic and odontome associated variant. The anterior region is commonly involved followed by the premolar and molar area. In contrast, all reports of the ameloblastomatous type involved the posterior mandible except 1 which occurred in the maxilla.^[14]

Patients with simple CCOT usually report an asymptomatic slowly growing swelling with a duration ranging from 6 months to 25 years.^[6] The size of the lesion usually ranges from 2 to 4 cm. Expansion of the labial or buccal cortical plate invariably occurs usually sparing the lingual cortical plate.^[21] In contrast, in abovementioned case reports of ameloblastomatous CCOT, patients have usually reported a symptomatic or asymptomatic fast-growing swelling and expansion of both the buccal and lingual cortical plates was invariably noted in all cases without perforation of the cortical plates.

Radiographically, the appearance of ameloblastomatous CCOT ranges from mostly a well-defined unilocular to multilocular radiolucency usually in association with an impacted tooth as discussed in these case reports. Some of them have demonstrated root resorption and divergence of adjacent teeth. On the other hand, simple cystic CCOT generally appears as a unilocular lesion with a well-defined margin. The presence of calcifications, which are observed in about half of them, is the most important radiographic feature for the diagnosis of CCOT.^[20],[21]

The histopathological features of a classic calcifying cystic odontogenic tumor include the presence of cystic lumen with a fibrous capsule and a lining of odontogenic epithelium. The basal layer is well defined and made up of ameloblast-like columnar cells, an overlying layer often many cells thick that may resemble the stellate reticulum of the enamel organ is seen. The characterestic feature of this lesion is the presence of masses of “ghost cells” which may be located within the epithelial lining or in the fibrous capsule.^[22]

The epithelial lining of the CCOT has the ability to induce the formation of mature dental tissues in the adjacent connective tissue wall and odontoma and ameloblastoma are commonly associated odontogenic tumor found associated with the cyst.

All recent histological classifications have established a category for the variant of CCOT associated with ameloblastoma. The classification advocated by Hong et al. has two categories for CCOT associated with ameloblastoma: The ameloblastomatous cystic variant and the neoplastic variant associated with ameloblastoma. The former is characterized by a unicystic structure in which the lining epithelium shows unifocal or multifocal intraluminal proliferative activity that resembles ameloblastoma, but that also contains isolated or clustered ghost cells and calcification. The latter is called ameloblastoma arising from CCOT (ameloblastoma ex CCOT). It is characterized histopathologically as comprising few or no ghost cells with calcification observed in the transformed ameloblastomatous epithelial portion, while the cyst lining of the epithelium contains a considerable number of ghost cells and calcifications. Also in contrast to ameloblastomatous CCOT, in ameloblastomata ex CCOT, Vickers and Gorlin criteria for ameloblastoma like cells can be easily identified.^[23],[24]

The treatment of ameloblatomatous CCOT has ranged from simple enucleation to hemimandibulectomy.

As observed in these case reports, if CCOT is associated with an ameloblastoma, its clinical behavior and radiological appearance is different, similar to that of an ameloblastoma, not of a CCOT. Thus, the prognosis is less certain for this form of CCOT, and a case report also presented with recurrence after 1 year of conservative management. To conclude, ameloblastomatous CCOT appears to be a more aggressive variant of simple CCOT both clinically and radiographically and needs to be managed accordingly.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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