Calcifying Cystic Odontogenic Tumor: A Case Report and Review

Case Report
DOI: 10.17354/cr/2015/09
Calcifying Cystic Odontogenic Tumor: A Case Report
and Review
Renjith George1, Preethy Mary Donald2, B Sabarinath3
Assistant Professor, Department of Oral Pathology, Faculty of Dentistry, Melaka-Manipal Medical College, Melaka, Malaysia, 2Assistant Professor,
Department of Oral Medicine and Oral Diagnosis, Faculty of Dentistry, Melaka-Manipal Medical College, Melaka, Malaysia, 3Associate Professor,
Department of Oral and Maxillofacial Pathology, Meenakshi Ammal Medical College, Chennai, Tamil Nadu, India
1
Calcifying cystic odontogenic tumor (CCOT) are uncommon benign slow-growing cysts of developmental origin partly exhibiting
characteristics of a neoplasm. Until date, the exact behavior of CCOT had been enigmatic and still the lesion portrays a platform to be debatable.
The lesion has been reviewed and readdressed from the date first reported about its diverse clinical and histopathological features. The name
of the lesion itself has undergone extensive review for the same reason. We report a case of calcifying odontogenic cyst in the mandible with
a review of the literature. This paper also highlights the update on nomenclature and classification.
Keywords: Cyst, Calcifying, Gorlin, Odontogenic
INTRODUCTION
Calcifying odontogenic cyst (COC) which is also known
as Calcifying cystic odontogenic tumor (CCOT) is defined
as a mixed radiolucent-radiopaque lesion of the jaws with
features of both a cyst and a solid neoplasm; characterized
microscopically by an epithelial lining showing a palisaded
layer of columnar basal cells, presence keratinization,
dentinoid, and calcification.1 It is a hybrid lesion of the jaw
exhibiting radiolucency and radiopacity showing cystic and
solid neoplastic characteristics. The cyst often resembles
features of ameloblastoma and may contain ghost cells,
tooth like material and calcified deposits.2
The classification and nomenclature of this lesion underwent
revisions many times from the day it has been reported, due
to its characteristic features and behavior. Here, we report a
case of CCOT and literature review highlighting the update
on nomenclature and classification.
CASE REPORT
A 52-year-old female patient reported with a swelling in the
anterior segment of the mandible. The swelling is present
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since 2 years and remained relatively of the same size.
She was asymptomatic, and her medical history was not
relevant. A firm, well-circumscribed, solitary, non-tender
swelling was seen in the right anterior mandibular region
occupying the labial and buccal vestibule measuring about
3 cm × 2 cm in diameter and extending anteriorly till the
midline and posteriorly till the first premolar (Figure 1).
Submandibular lymph nodes were not palpable. Overlying
mucosa is normal in color. Grade I mobility was seen with
teeth #32, #31, #41, #42 and #43.
Panoramic radiograph revealed a well-circumscribed
unilocular radiolucency with tiny radio-opaque flecks within
it, extending from the apical region of #31-44 (Figure 2). The
lesion was surrounded by a sclerotic margin. Based on the
clinical and radiographical features, differential diagnosis
of calcifying epithelial odontogenic tumor, ossifying
fibroma and CCOT were assigned. Radiographic features
suggestive of odontoma or adenomatoid odontogenic
tumor were not present. Fine needle aspiration cytology
was inconclusive. Incisional biopsy revealed a cystic lesion
showing lining of proliferative odontogenic epithelium
similar to ameloblastoma (Figure 3). Few cells are cuboidal
and stellate reticulum like cells are seen superficial to
ameloblast like cells. Excisional biopsy showed cystic lumen
with proliferating epithelium with areas of dystrophic
calcifications (Figure 4). Numerous large, homogenously
eosinophilic cells of varying sizes with pyknotic nuclei
suggestive of “ghost cells” were seen. Few of the ghost
cells stained with hematoxylin were suggestive of ghost cell
undergoing calcification (Figure 5). Adjacent to the ghost
Corresponding Author:
Dr. Renjith George, Department of Oral Pathology, Faculty of Dentistry, Melaka Manipal Medical College, Melaka - 75150, Malaysia.
Phone number: +60-111-8636120, Fax: +60-62896669. E-mail: drrenjithgeorgep@gmail.com
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George, et al.: Calcifying Cystic Odontogenic Tumor
Figure 1: A firm, well circumscribed, solitary, non-tender swelling was seen in
the right anterior mandibular region occupying the labial and buccal vestibule
Figure 4: Excisional biopsy showing cystic lumen with proliferating epithelium
with areas of dystrophic calcifications
Figure 2: Well circumscribed unilocular radiolucency with tiny radio-opaque
flecks within it, extending from apical region of #31 to #44
Figure 5: Ghost cells stained with hematoxylin were suggestive of ghost cell
undergoing calcification
suggestive of dentinoid material beneath the epithelial
lining. Proliferating strands and cord of ameloblastomatous
cells were not observed in the wall, ruling out the
proliferative variant dentinogenic ghost cell tumour
(DGCT). The lesion was diagnosed histopathologically as
simple cystic, non-proliferative CCOT and it was surgically
enucleated under general anesthesia. The follow-up was
done till 2013 and recurrence was nil.
DISCUSSION
Figure 3: Cystic lesion showing lining of proliferative odontogenic epithelium
similar to ameloblastoma with cuboidal and stellate reticulum like cells superficial
to ameloblast like cells
cells, irregular basophilic masses suggestive of dystrophic
calcification were also seen. The connective tissue showed
numerous endothelial lined blood vessels and mixed
inflammatory cell infiltrate, predominantly lymphocytes.
Within the capsule, some areas show eosinophilic masses
IJSS Case Reports & Reviews | January 2015 | Vol 1 | Issue 8
A wide span of descriptive terminologies have been assigned
to address this lesion till date due to the diverse behavior
of calcifying odontogenic cyst (COC). Gorlin initially called
COC as Gorlin’s cyst and was termed as keratinizing and
COC by Gold in 1963. Gorlin reported that COC may be the
oral analogue of dermal calcifying epithelioma of Malherbe.3
Precedently, the first author to publish COC was Rywkind
and later on he termed it as cholesteatoma of jaw.4,5 Table 1
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George, et al.: Calcifying Cystic Odontogenic Tumor
Table 1: Evolution of nomenclature of CCOT
1962 ‑ Gorlin et al. identified the lesion as a distinct pathological entity
1963 ‑ Gold named the lesion as KCOC
1972 ‑ Fejerskov and Krog used the term CGCOT ‑ monistic concept
1975 ‑ Freedman et al. introduced the term CCOT ‑ monistic concept
1981 ‑ Praetorius and Ledesma‑Montes suggested (COC ‑ cystic
type) and (DGCT ‑ neoplastic type) ‑ based on the dualistic concept
1986 ‑ Ellis and Shmookler suggested EOGCT
1990 ‑ Colmenero et al. suggested OGCT
1992 ‑ WHO classification ‑ According to Kramer and Pindborg from
1992 and the majority of the authors favored the use of the term
COC and described it as a cystic or neoplastic‑like odontogenic
pathological lesion of the jaw and classified it as a benign
odontogenic tumor
1994 ‑ Hirshberg et al. Considered COC associated with an
odontoma as a separate entity and suggested the name
odontocalcifying odontogenic cyst
1998 ‑ Toida named the entity as CGCOC
2005 ‑ The WHO Classification of odontogenic tumors re‑named this
entity as CCOT, the benign solid type was referred to as DGCT and
the cases previously reported as OGCC and malignant EOGOCs were
re‑named by WHO as GCOC
KCOC: Keratinizing and calcifying odontogenic cyst, CGCOT: Calcifying ghost cell
odontogenic tumor, CCOT: Cystic calcifying odontogenic tumor, COCt: Calcifying
odontogenic cyst, DGCT: Dentinogenic ghost cell tumor, EOGCT: Epithelial
odontogenic ghost cell tumor, OGCT: Odontogenic ghost cell tumor,
CGCOC: Calcifying ghost cell odontogenic cyst, OGCC: Odontogenic ghost cell
carcinoma, GCOC: Ghost cell odontogenic carcinoma
enlist the history of nomenclature of this lesion.3,6-15 The term
COC was given by World Health Organization (WHO) in
1992 and referred it a tumor like odontogenic cyst of the jaws
and was categorized under benign odontogenic tumors but
continued to use the term COC. COC is generally considered
to be a cyst, yet many researchers choose to address it as
a neoplasm, which led to sub classify the variants of the
lesion into cystic and neoplastic variants. In 2005, WHO
addressed the cyst as CCOT (SNOMED code 9301/0) and
DGCT (SNOMED code 9302/0) representing the cystic and
neoplastic variants respectively.16
The CCOT may prevail as an intrabony or extrabony form.
The age of the patient may vary from first to ninth decade
and thus far no gender predilection has been reported.17-19
Distribution of cases based on site for maxilla and mandible
are comparable. Extrabony CCOTs most commonly occur
as a swelling in the incisor-canine region calibrating
up to 4 cm and are mostly asymptomatic. They usually
have the appearance of well-delineated swellings having
smooth surface with a pink to reddish hue.20 Extra-osseous
CCOTs may reveal a shallow depression in the bone, and
occasionally displacement of adjacent teeth was observed.
Largely, intraosseous CCOTs are identified as unilocular
radiolucencies with a well-delineated border. 50% of
cases exhibit varying flecks of radiopacity. In most of the
cases, this entity reveals root resorption along with root
divergence and <25% of cases may show an association
with an unerupted tooth.2,15,16
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In the early stages of formation, CCOT may have little or no
mineralization and therefore may present as radiolucencies.
As the lesion matures, calcifications occur and appear as
well-circumscribed, mixed radiolucent-radiopaque masses.
Dense opacities are associated with complex odontome.
Radiologically, three basic patterns of radiopacities are
identified particularly salt and -pepper pattern of flecks,
fluffy cloudlike pattern throughout, and a “new moon” -like
configuration with crescent-shaped radio-opacity on one
side of the radiolucency.21 The differential diagnosis in
these instances includes dentigerous cyst, odontogenic
keratocyst (OKC), and ameloblastoma. In later stages when
a mixed radiolucent-radiopaque appearance is present,
the differential diagnosis would include adenomatoid
odontogenic tumor, a partially mineralized odontoma,
calcifying epithelial odontogenic tumor and ameloblastic
fibroodontoma. In the early stages of formation, differential
diagnosis can be dentigerous cyst, OKC, and ameloblastoma
as they have little or no mineralization and therefore may
present as radiolucencies.22
Histopathologically, CCOT reveals an odontogenic
epithelium with columnar basal cells, upper layers show
stellate reticulum like cells and a fibrous capsule. The
lesion differs histologically from the odontogenic cysts
and epithelial tumors such as ameloblastoma, but could
be similar to the Pindborg’s tumour.22 Ghost cells, tooth
like material and calcifications are also seen scattered in
the epithelium The mechanism of formation of ghost cells
is controversial; it may represent coagulative necrosis or a
form of normal or aberrant keratinization of odontogenic
epithelium. Some theories reveal an underlying ischemic
process, which may result in squamous metaplasia and
later tends to calcify.23 These eosinophilic ghost cells can be
a nuclear or with pyknotic nuclei and occasionally, ghost
cells tend to calcify. The dystrophic calcification of the
keratin may induce a foreign body response in the fibrous
capsule and may mimic pilomatricoma of skin.19,22 Masses
of ghost cells may coalesce together to form large sheets
of amorphous material. Juxta-epithelially, an eosinophilic
material resembling dentin may be evident owing to the
inductive effect by the odontogenic epithelium on the
adjacent mesenchymal tissue.24,25 However, these ghost
cells are not exclusively seen in CCOT, but are also seen
in other odontogenic tumors like odontoma, ameloblastic
fibro-odontoma and ameloblastic odontomas.18
In our case, the lesion appeared as a well-circumscribed
unilocular radiolucency with tiny radiopaque flecks within
it. Correlating with the clinical features and radiographic
findings, Pindborg’s tumor, CCOT and ossifying fibroma
was assigned. Though the age of the patient is well above
the most prevalent second decade, the slow growth and
characteristic radiographic features excluded other old
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George, et al.: Calcifying Cystic Odontogenic Tumor
age lesions like ameloblastoma. Absence of the associated
impacted tooth and also the age of the patient ruled out
adenomatoid odontogenic tumor and dentigerous cyst
which fits in well with the location.
CONCLUSION
Considering the debatable nomenclature of the lesion it
is advisable to conduct more studies to explore the finer
details so as to propose a classification, which is universally
acceptable.
13.
14.
15.
16.
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How to cite this article: George R, Donald PM, Sabarinath B. Calcifying
cystic odontogenic tumour: A case report and review. IJSS Case Reports &
Reviews 2015;1(8):28-31.
Source of Support: Nil, Conflict of Interest: None declared.
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