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• Dualistic concept: Some authors prefer to consider COC
as lesion existing in two forms either cyst or neoplasm.
• Monistic concept: others like to regard the lesion as a
tumor with a marked tendency toward cystic
• Gorlin: described first as Calcifying odontogenic cyst in
• Gold: Keratinizing calcifying odontogenic 1963
• Prætorius: proposed a widely used classification as one of
the first to suggest that ghost cell lesions comprise a
spectrum between true (developmental) cysts and solid
• Shear: Odontogenic ghost cell ameloblastoma 1994
• WHO calcifying cystic odontogenic tumour 2005
After 2005 WHO classification,
praetrius and Ledesama
changed the name.
• For the cystic type:
calcifying cystic odontogenic
• For the newplasm type:
dentinogenic ghost cell tumor
In 2005 the WHO authors also chose to classify all ghost cell lesions as neoplasms,
with little evidence besides the fact that ghost cell lesions occupy a spectrum
from completely cystic to solid growths of cells
They described three basic variants of the cystic lesions;
• A cyst with a moderate amount of mural proliferation of epithelium with limited
amounts of dentinoid.
• A cyst in association with an odontoma
• A cyst with extensive ‘‘ameloblastoma-like’’ proliferation in the wall or lumen of
They divided their tumors into (CCOT), (DGCT) and ghost cell odontogenic
carcinoma (GCOC). They further subdivided the CCOT in four distinct types;
• Type 1: simple cystic
• Type 2: CCOT associated with an odontoma
• Type 3: with ameloblastomatous-like proliferation
• Type 4: associated with other benign odontogenic tumors other than
• In chapter 6 of the WHO book, CCOT was
defined as a benign cystic neoplasm of
odontogenic origin, characterized by an
ameloblastoma-like epithelium with ghost cells
that may calcify
• DGCT was defined as a locally invasive
neoplasm characterised by ameloblastoma-like
islands of epithelial cells in a mature connective
• Aberrant ghost cell keratinization may be found
in association with varying dysplastic dentin (so
called dentinoid materials) amounts
• The previously reported cases named
odontogenic ghost cell carcinoma and
malignant epithelial odontogenic ghost cell
tumours were re-named by the WHO as ghost
cell odontogenic carcinoma (GCOC).
In Ledesma-Montes et al., (2008) study
• CCOT cases were divided into four sub-types:
• (i) simple cystic;
• (ii) odontoma associated;
• (iii) amelo- blastomatous proliferating;
• (iv) CCOT associated with benign odontogenic tumours other than odontomas.
the CCOT is
shown in which
Classification of the ghost cell odontogenic tumours studied
Calcifying cystic odontogenic tumour (CCOT).
type 1. Simple cystic CCOT.
type 2. Odontoma-associated CCOT
type 3. Ameloblastomatous proliferating CCOT
type 4. CCOT associated with benign odontogenic tumours
Dentinogenic ghost cell tumour (DGCT)
type 1. Central, solid, aggressive variant DGCT
type 2. Peripheral, less aggressive variant
Ghost cell odontogenic carcinoma (GCOC).
present either as central
(85%) or peripheral lesion
(15%). It shows bimodal age
of occurrence commonly
presenting in second and
seventh decade of life.
predilection towards any
gender and occurs in equal
frequency in either of the jaw
bones, anterior to the first
molar in the incisor-canine
expansion is the most
common presentation of the
central lesions, while sessile
or pedunculated smooth
surfaced mass are features of
• COC is predominantly an intraosseous lesion, although in 13% to 30% of
reported cases, COC has manifested as a peripheral lesion. About 65% of cases
are found in the incisor or canine areas. Mean age is 33 years, and most cases
are diagnosed in the second and third decades of life.
• However, COCs that are associated with odontomas tend to occur in younger
patients (mean age, 17 years). Central COC is usually a unilocular, well-defined
radiolucency, although the lesion may occasionally appear multilocular.
• Extraosseous COCs are usually localized sessile or pedunculated gingival masses
with no distinctive clinical features, and can resemble common gingival
fibromas, gingival cysts, or peripheral giant cell granulomas.
• They appear as variably sized odontogenic epithelial islands in a fibrous stroma
composed of peripheral palisading columnar cells and central stellate reticulum,
reminiscent of ameloblastoma. However, ghost cell nests are present within
• Radiopaque structures within lesions, either irregular
calcifications or toothlike densities, are present in
about one third to one half of cases. The radiolucent
lesion is associated with an unerupted tooth, usually a
canine, in approximately one third of cases.
• the central lesion appear as unilocular or sometime
multilocular radiolucency with or without calcified
• Size and opacity of the calcified structure varies,
sometime occupying the entire lesional area.
• may be associated with an odontoma (24-35%) or an
impacted tooth, most commonly the canine (10-32%).
• microscopic features of are characteristic:
Cyst epithelium is usually thin and tends to be detached easily, but is focally
thickened by keratinized epithelial cells and ghost cells. Hyperplastic basal cells
often grow into fibrous cyst walls, resulting in daughter cysts.
Unlike other odontogenic cysts, COCs may contain highly-differentiated lining
epithelium composed of columnar cells or stellate reticulum-like cells associated
with ghost cells, and undifferentiated lining epithelium resembling reduced
• Anucleated epithelial cells with retention of cellular outline are present either in
the epithelial lining or connective tissue is a characteristic finding and are called
• Individual ghost cells may fuse together to form large sheets of amorphous
eosinophilic structure on which calcification may occur.
• Irregular masses of calcified structure suggestive of dysplastic dentin are present
in association with basal layer denoting the inductive nature of the odontogenic
Calcifying odontogenic cyst (COC) and calcifying cystic odontogenic tumor (CCOT)
photomicrographs. COC, cystic epithelium is keratinized and produces irregular
calcifications and aberrantly keratinized ghost cells
The CCOT is a cystic lesion in which the epithelial
• Sometimes, variable quantities of dentinoid or dentin-like material are laid down
adjacent to the epithelial lining and sometimes dental hard tissues resembling
an odontoma are found. In some instances, melanin or clear cells could be
CCOT type 1
simple cystic CCOT.
• This is a cystic lesion with features identical to
the mentioned definition except for the
presence of an associated odontoma.
CCOT type 2
• This is similar to the CCOT type 1, but dentin
enamel, cementum and pulpal tissues are
present in the form of a complex or compound
CCOT type 3
• This is a cystic lesion with features similar to
those described above. It is characterized by the
presence of intraluminal or capsular plexiform
growths similar to those seen in the plexiform
variant of the cystic ameloblastoma. Sometimes
follicles simulating solid ameloblastoma can be
seen in the connective tissue capsule.
tumours other than
• Certain cases are similar to CCOT type 1, but
there are also areas resembling ameloblastic
fibroma, ameloblastic fibroodontoma,
adenomatoid odontogenic tumour or
• is defined as a solid neoplastic growth formed by
groups and islands of epithelial cells showing an
ameloblastoma-like basal cell layer that sometimes
shows nuclear polarization.
• In the central part of the ameloblastomatous
islands, tissue resembling the stellate reticulum of
the enamel organ can also be found.
Characteristically, it contains variable quantities of
dentin-like material in the surrounding
connective tissue and in close contact with the
epithelial islands. Also, groups of ghost cells within
the epithelial islands can be found. Two variants were
identified: aggressive central and non-aggressive
peripherally located tumours.
Dentinogenic ghost cell tumor photomicrographs. The
polygonal epithelial strands are proliferative in the vicinity of
eosinophilic dentinoid materials (D).
• was diagnosed in terms of the 2005WHO
guidelines. It is an uncommon malignant
neoplasm that exhibits prominent mitotic
activity, nuclear atypia and cellular
pleomorphism, groups of ghost epithelial cells,
necrosis, sometimes scarce mineralised or
dentin- like material are found and has an
infiltrative growth pattern. This neoplasm
presents locally aggressive and destructive
behaviour and at times, metastatic deposits
can be found.
• Malignant transformation rate
• From 113 CCOTs, one case developed to a malignant
• neoplasm (0.9%).
• Less than 30 cases were reported
• Previous reports indicate GCOTs have wide neoplastic potential.
• CCOT is a cystic, painless, slowly growing tumor that commonly presents as a
well-defined radiolucent or combined lesion that rarely recurs, whereas DGCT is
aggressive and recurrences may be expected, and GCOC is in actuality a
• Furthermore, the term GCOT is useful for describing a solid neoplastic COC
variant characterized by ameloblastomatous epithelial components accompanied
by abundant ghost cell clusters and dentinoid materials
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