CASE REPORT

Odontoma associated with calcifying cystic odontogenictumor in deciduous dentition: case report

Marcelo Lupion Poleti & Bruno Gomes Duarte &

Vanessa Soares Lara & Patrícia Freitas-Faria &

Izabel Regina Fischer Rubira-Bullen &

Eduardo Sanches Gonçales

Received: 26 November 2011 /Accepted: 21 April 2012 /Published online: 13 May 2012# Springer-Verlag 2012

AbstractBackground Initially described by Gorlin et al. in 1962, thecalcifying cystic odontogenic tumor (CCOT) may be asso-ciated with unerupted teeth, ameloblastomas, adenomatoidodontogenic tumors, and, in many cases, with odontomas. Itis rare in patients in the first decade of life, particularlyinvolving deciduous teeth. Surgery is the treatment ofchoice, with low recurrence rates.Case report We present a clinical case of CCOT associatedwith odontoma and a missing deciduous tooth in a 3-year-old female patient. The lesion was removed under generalanesthesia. The patient has been followed up for 1 year, andno recurrence was found. This appears to be the first reportin such a young age.

Keywords Calcifying cystic odontogenic tumor . Calcifyingodontogenic cyst . Odontogenic tumor . Gorlin cyst

Background

In accordance with the latest classification of odontogenictumors of the World Health Organization (WHO) [1], thecalcifying cystic odontogenic tumor (CCOT), previouslydesigned as calcifying odontogenic cyst [2], is a benign cysticneoplasm of odontogenic origin, characterized by anameloblastoma-like epithelium with ghost cells [1, 3]. CCOT

is an uncommon lesion representing 2 % of all odontogenictumors [4, 5], equally affecting patients of both genders andshowing similar frequency in the maxilla and mandible, with-out race predilection [1, 4, 6]. CCOT is a disorder with a greatvariety of clinical and histological behaviors [3, 4]. Micro-scopically, the CCOT is composed of a cystic cavity lined byodontogenic epithelium, usually with similar features to ame-loblastoma and often exhibiting extensive luminal and intra-mural proliferation [1, 4, 5, 7]. The typical appearance is thepresence of epithelial cells without nuclei (ghost cells), whichmay also calcify [1, 4, 5, 8].

CCOT may be seen in any decade of life [1, 4, 9];however, it is more common in the age range from 20 to60 years [10]. In rare cases, CCOT has been seen in pedi-atric patients, with very rare cases involving deciduous teeth[8, 11].

The purpose of the present paper was to report a rare caseof CCOT associated with an odontoma in a 3-year-oldfemale patient, affecting the deciduous maxillary canine.The case is discussed to call the attention of orthodontistsand pedodontists when there is absence or delayed eruptionof deciduous teeth.

Case report

A female child, aged 3 years, was referred to our clinic forevaluation of clinical absence of the deciduous maxillaryleft canine. The medical history was not contributory to thediagnosis. She was a perfectly healthy child. Intraoralexamination showed cortical bone expansion, absence ofthe deciduous maxillary left canine (DMxLC), and integrityof the mucous membrane (Fig. 1). The periapical radiographrevealed absence of DMxLC and its association withnumerous radiopacities mimicking denticles, circumscribed

M. L. Poleti :B. G. Duarte :V. S. Lara : P. Freitas-Faria :I. R. F. Rubira-Bullen (*) : E. S. GonçalesDepartment of Stomatology, Bauru School of Dentistry,University of São Paulo,Alameda Octávio Pinheiro Brisolla, 9-75,17012-901 Bauru, São Paulo, Brazile-mail: izrubira@fob.usp.br

Oral Maxillofac Surg (2013) 17:77–80DOI 10.1007/s10006-012-0333-x

by a radiolucent zone (Fig. 2). The presumptive diagnosiswas made of a compound odontoma. The treatment pro-posed was surgical removal of the lesion. Due to the age andextent of the lesion, surgery was performed under generalanesthesia with nasotracheal intubation. Initially, an osteot-omy was performed for exposure of the lesion, which wasthen removed by surgical enucleation, along with theDMxLC and multiples denticles (Fig. 3).

Microscopic examination of sections revealed a cysticlesion lined by a thin epithelium with a thickness of two-to three-cell layers (Fig. 4). The epithelium merged withfocal thickenings, which presented many central cellsresembling stellate reticulum, as well as numerous ghostcells, sometimes calcified. The fibrous wall showed variablequantities of odontogenic epithelium islands, commonly

with ghost cells and dentin-like material (Fig. 5). Enamelmatrix, reduced enamel epithelium, and dentin, sometimeswith dentinoid and pulp tissues, were present in the form ofa compound odontoma (Fig. 6). In addition, the fibrouscapsule exhibited a cellular ectomesenchymal tissue thatresembled a dental papilla, often presenting a peripherallayer of odontogenic epithelium similar to either internaldental epithelium or ameloblasts. Also, in some sections,ameloblastoma-like odontogenic epithelium islands wereembedded in the dental papilla, mimicking ameloblasticfibroma. Considering these morphological findings, a diag-nosis of odontoma-associated calcifying cystic odontogenictumor was made. Postoperative healing was uneventful, andthere were no signs of recurrence after 1 year of follow-up.

Discussion

The benign neoplasia CCOTwas first described by Gorlin etal. [12–14], and in 1981, Prætorius et al. [15] proposed aclassification, which was widely used and divided intocystic and neoplastic types. The WHO publication on odon-togenic tumors in 1992 [2] reported this tumor as a cysticlesion arising from the odontogenic epithelium with odon-togenic ectomesenchyme. For predominantly solid lesions,the term proposed was dentinogenic ghost cell tumor orodontogenic ghost cell tumor [2]. Recently, Prætorius andLedesma-Montes established as a principal nomenclaturethe term “calcifying cystic odontogenic tumor” for the cysticlesion, and synonyms were listed as keratinizing and calci-fying odontogenic cyst, Gorlin cyst, and calcifying odonto-genic cyst [1]. The solid variant of CCOT was nowrecognized as a distinct and separate pathological entity,according to the last WHO classification, and definitivelydenominated as dentinogenic ghost cell tumor [1].

Microscopically, CCOT is a cystic lesion in which theepithelial lining shows a well-defined basal layer of colum-nar cells and an overlying layer often resembling the stellatereticulum. Masses of ghost epithelial cells are seen in theepithelial cyst lining or in the fibrous capsule and maybecome calcified [2]. Proliferation of odontogenic epitheli-um in the adjacent connective tissue and dysplastic dentinmay be observed [1].

The CCOT is a rare pathological condition and may bediagnosed in any decade of life [1, 4, 6, 13, 16–18], yet thepeak prevalence seems to be between 20 and 60 years [10,17, 19]. Iida et al. [13] found higher prevalence in patientsaged 10 to 19 years.

CCOT involving pediatric patients is extremely rare, withonly two reported cases [8, 11] found after thorough reviewof the English language literature. Reyes et al. [8] describeda CCOT on the buccal aspect related to a deciduous maxil-lary left canine in a 5-year-old girl, while Fernandes et al.

Fig. 1 Intraoral examination showing absence of the deciduous maxil-lary left canine

Fig. 2 Periapical radiograph showing absence of the deciduous max-illary left canine associated with numerous radiopacities mimickingdenticles, circumscribed by a radiolucent area

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[11] reported this tumor affecting one child in the age range0–9 years, from their study of 340 cases of odontogenictumors in a Brazilian population. To our knowledge, thepresent case represents the first clinical documentation ofthis odontogenic tumor diagnosed in a child aged 3 years.The case reported by Reyes et al. [8] involving a pediatricpatient was also associated with odontoma, similar to thepresent case. According to Hirshberg et al. [20], the anteriormaxillary region is the most common site for this tumorwhen associated with odontoma, which again is consistentwith the present case. However, according to Iida et al. [13],CCOT in the maxilla tends to be found in older patients.

The CCOT may be intraosseous or extraosseous, and theformer tumors present a higher incidence in the maxillaryregion (2:1), commonly in the posterior site. Conversely, the

majority of extraosseous cases are seen in the anterior region[1, 6, 7]. The intraosseous variant of CCOT may be seen as apainless, slowly growing lesion that may sometimes result inbuccal and lingual cortical expansion [4, 6, 8]. The radio-graphic appearance may show radiolucent areas, either uni-or multilocular, well defined, containing calcifications [1, 3–6,8, 9]. This lesion may be associated with unerupted teeth [5, 8]or other odontogenic tumor [3, 4, 7, 8, 10]. The extraosseousCCOT generally appears as pink, well-defined, and painlessgingival swellings or nodules measuring up to 4 cm in diam-eter [1, 6, 8]. In contrast to the literature, the present case wasintraosseous and located in the anterior region; similar to othercases, this tumor was associated with local intraoral corticalbone expansion and clinical absence of one tooth. However,what makes this case interesting is the missing deciduoustooth. Since the patient was very young, the injury was con-fused with a developing odontoma.

Fig. 3 Transoperative view.The well-encapsulated lesionwas successfully removed fromthe cavity

Fig. 4 Calcifying cystic odontogenic tumor with epithelial cyst liningcomposed of cuboidal or columnar basal cells, thick upper layers withmany cells resembling stellate reticulum of the enamel organ, and nu-merous ghost cells, some of which are calcified. The fibrous capsule iscomposed of connective tissue, presenting blood vessels (hematoxylinand eosin stain, HE; original magnification, ×100)

Fig. 5 Masses of ghost cells in the epithelial cyst lining. Some ghostcells, mainly in the periphery of masses, are calcified (HE; originalmagnification, ×400)

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The surgical removal of CCOT by enucleation or curet-tage has been adequate, and the recurrence is rare [1, 6, 8,13, 14]. Conservative treatment as marsupialization hasbeen described [9]. In this case, due to the young age ofthe patient and size of the lesion, surgical treatment wasconducted under general anesthesia, as also performed byReyes [8]. After 1-year follow-up, no evidence of recurrencewas found, in agreement again with the pediatric casereported by Reyes et al. [8]. However, further follow-upmust be continued at least for 10 years for possible recur-rences. In summary, pedodontists and orthodontists shouldbe alert to the possibility that the absence or delayed erup-tion of primary teeth may indicate the presence of odonto-genic tumors or cysts.

Conflict of interest No conflict exists.

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Fig. 6 Tooth-like structures composed of enamel, dentin, and pulp, in theform of a compound odontoma. Some ghost cells are found between thedental tissues. EM enamel matrix, D dentin, P pulp, GC ghost cells (HE;original magnification, ×40)

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