Open Journal of Stomatology, 2013, 3, 314-317 OJST
http://dx.doi.org/10.4236/ojst.2013.36052 Published Online September 2013 (http://www.scirp.org/journal/ojst/)
A unique case of a calcifying cystic odontogenic tumor
Shoko Gamoh1*, Yukako Nakashima1, Hironori Akiyama1, Kimishige Shimizutani1,
Takuro Sanuki2, Junichiro Kotani2, Koji Yamada3, Shosuke Morita3,
Kazuya Tominaga4, Masahiro Wato4, Akio Tanaka4
1Department of Oral Radiology, Osaka Dental University, Osaka, Japan
2Department of Anesthesiology, Osaka Dental University, Osaka, Japan
3Department of Oral and Maxillofacial Surgery, Osaka Dental University, Osaka, Japan
4Department of Oral Pathology, Osaka Dental University, Osaka, Japan
Received 23 June 2013; revised 23 July 2013; accepted 10 August 2013
Copyright © 2013 Shoko Gamoh et al. This is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
The calcifying odontogenic cyst was first reported by
Gorlin et al. in 1962. At that time, it was classified as
a cyst related to the odontogenic apparatus, although
it was later renamed as a calcifying cystic odonto-
genic tumor by the WHO calcification in 2005 due to
its histological complexity, morphological diversity
and aggressive proliferation [2]. Here, we describe a
case of a calcifying cystic odontogenic tumor in a 4-
year-old boy. The lesion was surgically removed, and
the histopathological examination revealed it to be a
cystic tumor with ghost cells, a stellate reticulum and
small amount of dentinoid tissue in the cystic wall.
Keywords: Calcifying Cystic Odontogenic Tumor;
Diagnostic Imaging; Computed Tomography
1. INTRODUCTION
The calcifying cystic odontogenic tumor (CCOT), which
is also known as a calcifying odontogen ic cyst (COC) or
Gorlin cyst, is a rare developmental lesion that arises
from the odontogenic epithelium [1]. Although it has
commonly been recog nized as a benign odon togenic cyst
since its original description by Gorlin et al. in 1962, this
pathologic entity encompasses a spectrum of clinical be-
haviors and histopathological features including cystic,
neoplastic and aggressive variants. As a result of this di-
versity, different classification schemes and nomencla-
tures for this type of lesion have been suggested. In 2005,
the World Health Organization Classification of Head
and Neck Tumors reclassified the lesion as an odonto-
genic tumor and gave it the name of “calcifying cystic
odontogenic tumor” (CCOT) [2].
CCOT is a rare lesion that represents about 2% of all
odontogenic pathological changes in the jaw [3-5]. It is
clinically characterized as a painless, slow-growing tu-
mor, which equally affects the maxilla and mandible, has
a predilection for the anterior region of the jaw and usu-
ally arises intraosseously, although it may also occur
extraosseou sly. It ha s a peak incidence during the second
and third decades on life, with a mean age of 30.3 years
and does not demonstrate any gender predilection [6].
The treatment of choice for cases of CCOT is conserva-
tive surgical enucleation. However, recurrence is fre-
quent, especially in neoplastic cases, such as dentinoge-
nic ghost cell tumor [7].
The typical histopathological features of CCOT in-
clude a fibrous wa ll and lin ing of odon toge nic epith eliu m
with columnar or cuboidal basal cells resembling ame-
loblasts. Stellate reticulum-like cells overlay the basal
cell layer, and “ghost cells”, which may occasionally be-
come calcified, are also situated in the cyst lining [8].
Radiographically, CCOT may appear as an unilocular
or multilocular radiolucent area with either well-cir-
cumscribed or poorly-defined margins that also may be
observed in association with unerupted teeth [3,7]. Calci-
fication is an important radiographic feature for the in-
terpretation of CCOT, but is detected in only approxi-
mately half of the reported cases [9].
This report describes a case of CCOT that occurred in
the maxilla of a 4-year-old Japanese boy.
2. CASE REPORT
A 4-year-old boy was referred to the Osaka Dental Uni-
versity Hospital by his home doctor due to 6-month his-
tory of asymptomatic swelling in the upper canine area.
However, at the time that his mother sought the check-up,
*Corresponding a uthor.

S. Gamoh et al. / Open Journal of Stomatology 3 (2013) 314-317 315
swelling had increased in size and his nose had been
bleeding profusely. He had no other concurrent diseases.
The clinical examination revealed the presence of a
firm and localized lesion, although the oral mucosa was
normal. His mother repo rted that his pregnancy and b irth
were normal. In addition, he had no history of traumatic
dental injury. The extra-oral examination revealed a dis-
crete facial asymmetry due to swelling in the middle of
the face that was close to the right nasal alar rim (Figure
1). Conventional radiographs showed a well-defined
unilocular, golf ball sized and round radiolucent lesion,
extending from tooth 12 to tooth 17 and representing the
inter-alveolar septum of the right upper canine and pre-
molar. The lesion included impacted tooth and contained
several calcified fragments (Figures 2(a) and (b)). Com-
puted tomography images revealed a well-defined, ex-
pansive lesion with thin cortical margins and containing
irregular radiopaque masses. No root divergency or de-
structive conditions were observed (Figures 3(a)-(c)).
The differential diagnosis involved analyzing the likely-
hood that the mass was a calcifying cystic odontogenic
tumor, calcifying epithelial odontogenic tumor, or ade-
nomatoid odontogenic tumor.
Under general anesthesia, an excisional biopsy was
carried out (Figures 4(a) and (b)), and the histopatho-
logical examination revealed the presence of cellular and
densely fibrous connective tissue. In the overlying layer,
cells were loosely and diffusely arranged together with
several “ghost cells”, some of which presented dystro-
phic calcification (Figures 5(a) and (b)). A microscopic
diagnosis of CCOT was made.
There are no signs of recurrence for a year and a half
since the complete removal of the tumor in October 2011
(Figure 6).
Figure 1. Front face photograph shows discrete
facial asymmetry due to swelling in the middle of
the face that is close to the right nasal alar rim.
(a)
(b)
Figure 2. Conventional radiographs. (a) Panoramic
and (b) posteroanterior radiographs showed a well-
defined unilocular radiolucent lesion associated with
radiopaque bodies in the right upper premolar area.
(a) (b)
(c)
Figure 3. Computed tomography images. (a) Axial view of CT
image, (b) coronal view of CT image, and (c) 3-dimensional
reconstructed image of CT image reveal a well-defined and
expansive lesion with thin cortical margins and irregular ra-
diopaque masses.
3. DISCUSSION
COC, a rare lesion first described by Gorlin et al. [1], has
recently been defined as calcifying cystic odontogenic
tumor (CCOT) by the World Health Organization (W HO)

S. Gamoh et al. / Open Journal of Stomatology 3 (2013) **-** 316
(a) (b)
Figure 4. Operation findings. (a) A site of operation. (b) The
extracted lesion 25 mm in diameter. The radiopaque masses
found on CT image revealed to be a calcified portion in the
mass.
(a) (b)
Figure 5. Histopathological findings. (a) The cystic wall is
lined by an ameloblast-like epithelium with columnar and cu-
boidal basal layer. Numerous ghost cells and calcified particles
are present within the epithelial lining (hematoxylin-eosin stain,
original magnification ×10). (b) Stellate reticulum cells are
observed within the epithelial lining (hematoxylin-eosin stain,
original magnification ×20).
Figure 6. A panoramic radiograph on a year and a half after
the operation showed no signs of recurrence.
due to its neoplastic behavior [2]. Alternative names
have also been used, such as “keratinizing and calcifying
odontogenic tumor” [10,11], “calcifying ghost cell odon-
togenic tumor” [12], “dentinogenic ghost cell tumor”
[13-15] and “epithelial odontogenic ghost cell tumor”
[16,17]. The existence of such a variety of terms reflects
both the wide clinicopathological spectrum of the for-
merly named COC and the confusion regarding its cystic
or neoplastic nature.
Most CCOTs, as demonstrated by our case, are as-
ymptomatic and often discovered incidentally on radio-
graphic examinations [18]. Because this lesion arises in
tooth-bearing areas of the jaws or gingival areas, CCOTs
are often located in a periapical or lateral periodontal
relationship to the adjacent teeth. Radiographically, they
are clearly-delineated and appear as unilocular or multi-
locular radiolucencies, with calcifications of variable
density noted in one-third to one-half of cases. CCOTs
can occur alone or in association with other odontogenic
tumors such as odontomas (20%), adenomatoid odonto-
genic tumors and ameloblastomas [18]. However, this
association is a challenge for diagnosis using only con-
ventional images, due to the presence of numerous over-
lapped images of anatomical structures of the jaw region .
Root resorption and divergency are common radiogra-
phic findings, and an association with an impacted tooth
occurs in approximately one-third of cases [19].
Enucleation is the treatment of choice for most in-
traosseous CCOTs and few recurrences have been re-
ported in the literature [2].
CCOT is an uncommon entity. Although rare, calcify-
ing epithelial odontogenic tumor and adenomatoid odon-
togenic tumor should be included in the differential di-
agnosis of jaw lesions. The chief danger is the losing of
permanent teeth [20]. Despite their unknown pathogene-
sis, CCOT should be treated conservatively, and preser-
vation of unerupted teeth is encouraged, especially when
intervention occurs at a young age.
In conclusion, this was an observed case of CCOT that
was associated with an impacted tooth. A satisfactory
esthetic outcome without sequ elae was obtained.
REFERENCES
[1] Gorlin, R.J., Pindborg, J.J., Clausen, F.P. and Vickers,
R.A. (1962) The calcifying odontogenic cyst—A possible
analogue of the cutaneous calcifying epithelioma of Mal-
herbe. An analysis of fifteen cases. Oral Surgery, Oral
Medicine, Oral Pathology, 15, 1235-1243.
doi:10.1016/0030-4220(62)90159-7
[2] Praetorius, F. and Ledesma-Montes, C. (2005) Dentino-
genic ghost cell tumour. In: Barnes, L., Eveson, J.W.,
Reichart, P. and Sidransky, D., Eds., World Health Or-
ganization Classification of Tumours. Pathology and Ge-
netics of Head and Neck Tumours. IARC Press, Lyon,
314.
[3] Chindasombatjaroen, J., Ka kimoto, N., Akiyama, H., Ku-
bo, K., Murakami, S., Furukawa, S. and Kishino, M. (2007)
Computerized tomography observation of a calcifying
cystic odontogenic tumor with an odontoma: Case report.
Oral Surgery, Oral Medicine, Oral Pathology, Oral Ra-
diology, and Endodontology, 104, e52-e57.
doi:10.1016/j.tripleo.2007.06.025
[4] Verbin, R. and Barned, L. (2001) Cysts and cyst-like
lesions of the oral cavity, jaws and neck. In: Barnes, L.,
Ed., Surgical Pathology of the Head and Neck, Vol. 3,
2nd Edition, Marcel Decker, New York, 1437-1555.
[5] Kamboj, M. and Juneja, M. (2007) Ameloblastoma Gor-

S. Gamoh et al. / Open Journal of Stomatology 3 (2013) 314-317 317
lin’s cyst. Journal of Oral Science, 49, 319-323.
doi:10.2334/josnusd.49.319
[6] Ledesma-Montes, C., Gorlin, R.J., Shear, M., Prae Torius,
F., Mosqueda-Taylor, A., Altini, M., et al. (2007) Inter-
national collabolative study on ghost cell odontogenic
tumours. Calcifying cystic odontogenic tumour, dentino-
genic ghost cell tumour, and ghost cell odontogenic car-
cinoma. Journal of Oral Pathology & Medicine, 37, 302-
308.doi:10.1111/j.1600-0714.2007.00623.x
[7] Sakai, V.T., Filho, C.E., Moretti, A.B., Pereira, A.A., Ha-
nemann, J.A. and Duque, J.A. (2011) Conservative sur-
gical treatment of an aggressive calcifying cystic odon-
togenic maxillary tumor in the young permanent dentition.
Pediatric Dentistry, 33, 265-269.
[8] Kler, S., Palaskar, S., Shetty, V.P. and Bhushan, A. (2009)
Intraosseous calcifying cystic odontogenic tumor. Jour-
nal of Oral and Maxillofacial Pathology, 13, 27-29.
doi:10.4103/0973-029X.48753
[9] Marques, Y.M.F.S., Botelho, T.L., Xavier, F.C.A., Ran-
gel, A.L., Rege, I.C.C. and Mantesso, A. (2010) Impor-
tance of cone beam computed tomography for diagnosis
of calcifying cystic odontogenic tumor associated to od-
ontoma. Report of a case, Medicina Oral Patologia Oral
y Cirugia Bucal, 1, ed3, e490-e493.
[10] Niikuni, T., Takigawa, T., Kawagoe, H., Kobayashi, A.
and Yokoi, S. (1966) A clinical case of keratinizing and
calcifying odontogenic epithelial tumor in the upper jaw.
Journal of Nihon University School of Dentistry, 8, 116-
122. doi:10.2334/josnusd1959.8.116
[11] Jones, J.H., McGowan, D.A. and Gorman, J.M. (1968)
Calcifying epithelial odontogenic and keratinizing odon-
togenic tumors. Oral Surgery, Oral Medicine, Oral Pa-
thology, 25, 465-469. doi:10.1016/0030-4220(68)90022-4
[12] Fejerskov, O. and Krogh, J. (1972) The calcifying ghost
cell odontogenic tumor or the calcifying odontogenic cyst.
Journal of Oral Pathology, 1, 273-287.
doi:10.1111/j.1600-0714.1972.tb01666.x
[13] Singhaniya, S.B., Barpande, S.R. and Bhavthanker, J.D.
(2009) Dentinogenic ghost cell tumor. Journal of Oral
and Maxillofacial Pathology, 13, 97-100.
doi:10.4103/0973-029X.57679
[14] Kumar, U., Vij, H., Vij, R., Kharbanda, J., Aparna, I.N.
and Radhakrishman, R. (2010) Dentinogenic ghost cell
tumor of the peripheral variant mimicking epulis. Inter-
national Journal of Dentistry.
doi:10.1155/2010/519494
[15] Li, B.B. and Gao, Y. (2008) Ghost cell odontogenic car-
cinoma transformed from a dentinogenic ghost cell tumor
of maxilla after multiple recurrences. Oral Surgery, Oral
Medicine, Oral Pathology, Oral Radiology, and Endodo-
ntology, 107, 691-695. doi:10.1016/j.tripleo.2009.01.008
[16] Lombardi, T., Felice, R.D. and Samson, J. (1999) Epithe-
lial odontogenic ghost cell tumour of the mandibular gin-
gival. Oral Oncology, 35, 439-442.
[17] Kim, H.J., Choi, S.K., Lee, C.J. and Suh, C.H. (2001)
Aggressive epithelial odontogenic ghost cell tumor in the
mandible: CT and MR imaging findings. American Jour-
nal of Neuroradiology, 22, 175-179.
[18] Zornosa, X. and Muller, S. (2010) Calcifying cystic odo-
ntogenic tumor. Head & Neck Pathology, 4, 292-294.
doi:10.1007/s12105-010-0197-z
[19] Buchner, A. (1991) The central (intraosseous) calcifying
odontogenic cyst: An analysis of 215 cases. Journal of
Oral and Maxillofacial Surgery, 49, 330-339.
doi:10.1016/0278-2391(91)90365-S
[20] Soares, E.C.S., Costa, F.W.G., Neto, I.C.P., Bezerra, T.P.,
Patrocinio, R.M.S.V. and Alves, A.P.N.N. (2011) Rare
hybrid odontogenic tumor in a 2-year-old child. Journal
of Craniofacial Surgery, 22, 554-558.
doi:10.1097/SCS.0b013e3182074616