| Abstract|| |
Calcifying odontogenic cysts (COCs) represent a heterogeneous group of lesions that exhibits a variety of clinical, behavioral, and histological features. COC or Gorlin's cyst is now classified by the World Health Organization as a tumor. Calcifying cystic odontogenic tumor (CCOT) is an uncommon developmental odontogenic lesion that demonstrates histopathologic diversity. Odontogenic tumors such as ameloblastoma have been reported to be associated with CCOT. In this paper, we report a case of ameloblastomatous CCOT in a 16-year-old girl with involvement of mandibular posterior region, which is an extremely rare histologic variant.
Keywords: Ameloblastoma, calcifying cystic odontogenic tumor, Gorlin's cyst
|How to cite this article:|
Muddana K, Maloth AK, Dorankula SP, Kulkarni PG. Calcifying cystic odontogenic tumor associated with ameloblastoma – A rare histological variant. Indian J Dent Res 2019;30:144-8
|How to cite this URL:|
Muddana K, Maloth AK, Dorankula SP, Kulkarni PG. Calcifying cystic odontogenic tumor associated with ameloblastoma – A rare histological variant. Indian J Dent Res [serial online] 2019 [cited 2021 Jul 28];30:144-8. Available from: https://www.ijdr.in/text.asp?2019/30/1/144/254501
| Introduction|| |
Calcifying odontogenic cyst (COC) is a unique and uncommon developmental odontogenic lesion first described by Gorlin et al. in 1962. It considered as a rare lesion and accounts for 1% of jaw cysts. To the best of our knowledge, till now, only 30 cases of CCOT associated with ameloblastoma have been reported. In 2005, the WHO reclassified COC as calcifying cystic odontogenic tumor (CCOT). It usually occurs as a slow-growing painless tumor, there is no predilection to either maxilla or mandible. However, some studies, have mentioned the mandible as the more commonly involved site. Mashhadi Abbas et al. in 2009 have reported a peak frequency in the second decade. Radiographically, CCOT may present as unilocular or multilocular radiolucencies with discrete, well-demarcated margin. The CCOT presents a wide range of histologic features, but most commonly a well-defined cystic lesion is found with a fibrous connective tissue wall lined by odontogenic epithelium. The most characteristic histopathologic feature is the presence of variable numbers of ghost cells within the epithelium component. They may also show ameloblastomatous proliferative activity intraluminally or intramurally. The neoplastic variant of CCOT shows a solid growth pattern consisting of ameloblastoma-like strands and islands of odontogenic epithelium infiltrating into mature fibrous connective tissue.
| Case Report|| |
A 16-year-old female patient presented to the department of oral medicine and radiology with the chief complaint of swelling in the right mandibular buccal vestibular region for 3 months. The swelling started as a small size and gradually increased to attain the present size. No history of pain and other associated signs and symptoms was present. Her medical history and dental history were noncontributory. Extraoral examination revealed a solitary swelling present on the lower right body of mandible, measuring 3 cm × 2 cm in diameter, which was nontender and bony hard in consistency. Intraorally, swelling was present in the right buccal sulcus causing vestibular obliteration, measuring 3 cm × 2 cm, extending from distal aspect of 43 to distal aspect of 46 with grade 1 mobility of 46. Mucosa over the swelling appeared to be normal.
Panoramic radiography revealed a well-defined radiolucency involving the right body of the mandible from tooth 43 to 46. The lesion measured about 3 cm × 6 cm and was oval. Resorption of root of 46 was also evident [Figure 1]. Axial section in computed tomography revealed the presence of a large unilocular radiolucency with the expansion of well-corticated right mandibular buccal and lingual cortical plates [Figure 2]. On the basis of history and clinical examination and radiological features, provisional diagnosis was given as infected odontogenic cyst.
|Figure 2: Computed tomography scan shows hypodense area, buccal cortical plate expansion on the right side of mandible|
Click here to view
To obtain a definite diagnosis, an incisional biopsy was taken from the buccal cortex between teeth #44 and #46 and sent for histopathological evaluation.
Histologic examination of the biopsy tissue revealed a cystic lesion lined by odontogenic epithelium. The lesion showed cystic lining containing basal tall columnar cells with reverse polarity of the nucleus. There is also the presence of suprabasal stellate reticulum-like cells with numerous ghost cells within the lining epithelium [Figure 3]. Connective tissue capsule consists of collagen fiber bundles and dense inflammatory cells. Based on the histological features, a diagnosis of infected CCOT was given.
|Figure 3: Cystic cavity lined by odontogenic epithelium with ghost cells|
Click here to view
The patient was treated surgically. Under general anesthesia, the right hemimandibulectomy was done in relation to 44–46 regions. Reconstruction was done by demineralized dried bone allograft with platelet-rich fibrinogen. Entire excisional specimen was sent for histopathological reevaluation.
Excisional biopsy tissue shows similar features of CCOT. In few areas, the cystic epithelium exhibited proliferation in the form of thin strands and cords [Figure 4]. Cords lined by tall columnar ameloblast-like cells and connected with stellate reticulum-like cells. Few ghost cells were present within the epithelium [Figure 5] Connective tissue shows the presence of dense collagen bundles, hemorrhagic areas, and inflammatory cell infiltrate. Based on the above findings, CCOT associated with ameloblastoma was given (according to Praetorius 2006). No immunohistochemical analysis was performed for the present case as histopathological evaluation is confirmatory for the diagnosis.
| Discussion|| |
The CCOT is an uncommon lesion that demonstrates considerable histologic diversity and presents variable clinical behavior. It shows both cystic and neoplastic variants. In view of this duality, many different terminologies have been applied to cystic and neoplastic variants. Several classifications of CCOT subtypes have been proposed, but most of them have limitations in separating cystic and neoplastic variant.
Different classifications are shown in [Table 1].,,,,
Ameloblastomatous CCOT is a rare variant because of the rarity of ameloblastomatous CCOT, and determination of the most common age, sex, and location of this lesion is difficult. According to Kallalli in 2017 and Mashhadi Abbas et al. in 2008, it seems that most of the patients are between 10-30 years old, and the tumor tends to involve the posterior region of the mandible., In the present case, the lesions involve mandibular posterior region in a 16-year-old patient.
Radiographically, the lesion appears as a well-defined, unilocular, or multilocular, often quite large radiolucency that may or may not contain varying amounts of radiopaque material associated with unerupted tooth. Root resorption or root divergence may also be observed. The present case showed unilocular radiolucency extending in the right side body of the mandible, extending from distal aspect of #43 to #46.
Ameloblastomatous CCOT microscopically resembles unicystic ameloblastoma, except for the ghost cells and calcification within the proliferative epithelium. The present case had similar microscopic findings. Histopathologically, our case has been diagnosed as CCOT associated with ameloblastoma due to the ghost cells in the ameloblastomatous epithelial islands, and it fits into the category of cystic calcifying ghost cell odontogenic tumor, as suggested by Toida. Or CCOT associated with ameloblastoma according to Praetorius 2006. Bharti et al., in 2010, suggested that if the COC was associated with an ameloblastoma, its behavior and prognosis would be like neoplasm rather than cyst.
There are few odontogenic lesions which are consider to be hisopathological mimicker of the CCOT associated with ameloblastoma, such as CCOT with odontoma, CCOT associated with ameloblastic fibroma.
However, CCOT associated with odontoma shows the presence of dentin-like hard tissue and pulp spaces histologically, which is not evident in the present case. CCOT associated with ameloblastic fibroma exhibits dental papilla-like mesenchyme along with numerous small odontogenic follicles, which is not seen in the present case.
| Conclusion|| |
CCOT associated with ameloblastoma is a rare histologic variant. There are very few cases reported with this variety of CCOT in the literature. Several authors have suggested that if CCOT is associated with an ameloblastoma, its behavior and prognosis will be that of an ameloblastoma, not of CCOT. Many cases with ameloblastomatous CCOT should be reported to understand its biological behavior. Careful postoperative observations are necessary for these types of cases.
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
Conflicts of interest
There are no conflicts of interest.
| References|| |
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.
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.
Li TJ, Yu SF. Clinicopathologic spectrum of the so-called calcifying odontogenic cysts: A study of 21 intraosseous cases with reconsideration of the terminology and classification. Am J Surg Pathol 2003;27:372-84.
Mashhadi Abbas FA, Moshref MA, Sargolzaie SA, Kargahi N. Ameloblastomatous calcifying cystic odontogenic tumor: A rare histologic variant. Shiraz Univ Dent J 2009;9:45-8.
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.
Ledesma-Montes C, Gorlin RJ, Shear M, Prae Torius F, Mosqueda-Taylor A, Altini M, et al.
International collaborative study on ghost cell odontogenic tumours: Calcifying cystic odontogenic tumour, dentinogenic ghost cell tumour and ghost cell odontogenic carcinoma. J Oral Pathol Med 2008;37:302-8.
Singh HP, Yadav M, Nayar A, Verma C, Aggarwal P, Bains SK. Ameloblastomatous calcifying ghost cell odontogenic cyst – A rare variant of a rare entity. Ann Stomatol (Roma) 2013;4:156-60.
Praetorius F, Hjørting-Hansen E, Gorlin RJ, Vickers RA. Calcifying odontogenic cyst. Range, variations and neoplastic potential. Acta Odontol Scand 1981;39:227-40.
Barnes L, Eveson JW, Reichart PA, Sidransky D. World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of the Head and Neck. Lyon: IARC Press; 2005.
Toida M. So-called calcifying odontogenic cyst: Review and discussion on the terminology and classification. J Oral Pathol Med 1998;27:49-52.
Hong SP, Ellis GL, Hartman KS. Calcifying odontogenic cyst. A review of ninety-two cases with re-evaluation of their nature as cysts or neoplasms, the nature of ghost cells and subclassification. Oral Surg Oral Med Oral Pathol 1991;72:56-64.
Shear M, Speight P. Cysts of the Oral and Maxillofacial Regions 4th
edition Blackwell- Munksgaard 2007. p. 101. (Praetorius F 2006, personal communication).
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.
Bharti CS. Calcifying odontogenic cyst – A case report and review of literature. JIDAT 2010;2:140-2.
Radheshyam C, Alokenath B, Kumar H, Abikshyeet P. Calcifying cystic odontogenic tumor associated with an odontome – A diverse lesion encountered. Clin Cosmet Investig Dent 2015;7:91-5.
Yoon JH, Kim HJ, Yook JI, Cha IH, Ellis GL, Kim J. Hybrid odontogenic tumor of calcifying odontogenic cyst and ameloblastic fibroma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98:80-4.
Dr. Keerthi Muddana
Department of Oral and Maxillofacial Pathology, Kamineni Institute of Dental Sciences, Sreepuram, Narketpally, Nalgonda - 508 254, Telangana
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]