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Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 421425

Contents lists available at ScienceDirect

Journal of Oral and Maxillofacial Surgery,


Medicine, and Pathology
journal homepage: www.elsevier.com/locate/jomsmp

Case Report

A case of a calcifying cystic odontogenic tumor with odontoma in a


5-year-old boy
Norifumi Moritani a, , Naoki Nakata a , Eiki Yamachika b , Tatsushi Matsumura a ,
Hitoshi Nagatsuka c , Seiji Iida a,b
a
Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences,
2-5-1, Shikata-cho, Kita-Ku, Okayama-Shi, Okayama 700-8558, Japan
b
Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University Hospital, 2-5-1, Shikata-cho, Kita-Ku, Okayama-Shi, Okayama
700-8558, Japan
c
Department of Oral Pathology and Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1,
Shikata-cho, Kita-Ku, Okayama-Shi, Okayama 700-8558, Japan

a r t i c l e i n f o a b s t r a c t

Article history: We report a case of calcifying cystic odontogenic tumor (CCOT) with odontoma developing in the decid-
Received 31 August 2015 uous dentition period, and present previously reported cases in Japan. A 5-year-old boy visited a dental
Received in revised form 14 January 2016 clinic because of swelling on his left mandibular buccal alveolar region, and was referred to our depart-
Accepted 31 March 2016
ment. Clinical examination revealed a 20-mm (in diameter), bone-like, hard mass on the left mandibular
Available online 4 May 2016
buccal gingiva at the rst deciduous molar region. A panoramic radiograph showed a 25 mm 20 mm,
unilocular, cystic radiolucent area from the left mandibular second deciduous molar to the rst molar
Keywords:
region. There were radiopaque tiny ecks at the upper side of the lesion. The clinical diagnosis was a
Calcifying cystic odontogenic tumor
Odontoma
benign tumor of the left mandible. Consequently, tumor enucleation was performed under general anes-
Deciduous dentition thesia. Histopathological diagnosis conrmed a CCOT associated with odontoma. Five years after surgery,
the left mandibular region has healed well, with no evidence of recurrence.
2016 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.

1. Introduction the deciduous dentition period in a 5-year-old boy, and discuss the
relevant literature.
Calcifying cystic odontogenic tumor (CCOT) is a disease display-
ing neoplastic potential and is characterized by calcication of the 2. Case report
epithelium and the presence of ghost cells. Although various sus-
ceptible ages have been reported, CCOT tends to occur frequently The patient is a 5-year-old boy. In November 2008, he visited a
in the young. However, cases of CCOT in patients aged <10 years dental clinic to undergo radiography for dental caries. These images
are rare; in particular, there is only one reported case in Japan of indicated the presence of a radiolucent area in the left mandible,
CCOT occurring in the deciduous dentition period [1]. We report and the patient was referred to our department for detailed exami-
our experience of a case of CCOT with odontoma developing in nation. He did not present with any particular past history or family
history of note. He had a height of 100 cm, weight of 15 kg, and
low body mass index, but good nutritional status. The external
orofacial appearance showed bilateral asymmetry. Regional lymph
nodes were without swelling or tenderness. The left mental nerve
Abbreviation: CCOT, calcifying cystic odontogenic tumor.
Asian AOMS: Asian Association of Oral and Maxillofacial Surgeons; ASOMP: Asian region did not demonstrate hypoesthesia. The internal oral cavity
Society of Oral and Maxillofacial Pathology; JSOP: Japanese Society of Oral Pathol- examination revealed a bone-like, hard mass 20 mm in diameter in
ogy; JSOMS: Japanese Society of Oral and Maxillofacial Surgeons; JSOM: Japanese the buccal alveolar region of the left mandibular second deciduous
Society of Oral Medicine; JAMI: Japanese Academy of Maxillofacial Implants. molar; however, no parchment-like appearance was observed.
Corresponding author. Tel.: +81 86 235 6697; fax: +81 86 235 6699.
A panoramic radiograph showed a 25 mm 20 mm, unilocular,
E-mail addresses: hachi70@md.okayama-u.ac.jp (N. Moritani),
n-nakata@md.okayama-u.ac.jp (N. Nakata), eikiyama@md.okayama-u.ac.jp
cystic radiolucent area from the left mandibular second decid-
(E. Yamachika), tatsushi@md.okayama-u.ac.jp (T. Matsumura), uous molar periapical region to the rst molar region. There
jin@md.okayama-u.ac.jp (H. Nagatsuka), iida-s1@cc.okayama-u.ac.jp (S. Iida). were radiopaque tiny ecks at the upper side of the area. The

http://dx.doi.org/10.1016/j.ajoms.2016.03.009
2212-5558/ 2016 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.
422 N. Moritani et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 421425

impacted left mandibular second premolar adjacent to the tumor,


the mandibular rst molar, and the mandibular second molar were
deviated. The left mandibular second deciduous molar adjacent to
the tumor demonstrated distal root resorption; however, there was
no mobility, and a vital reaction was observed (Fig. 1). Computed
tomography showed a unilocular, cystic radiolucent area from the
left mandibular second deciduous molar periapical region to the
distal root, with buccolingual mandibular protrusion, cortical bone
resorption, and cortical bone thinning. At the upper side of the cys-
tic radiolucent area, there was a tooth-like radiopaque area. The
size of the tumor was 25 mm 20 mm 19 mm (Fig. 2). Based on
the above ndings, we made a clinical diagnosis of a tumor of the
left mandible.

Fig. 1. Panoramic radiograph at the initial examination. A panoramic radiograph


showed a 25 mm 20 mm, unilocular cystic radiolucent area from the left mandibu-
lar second deciduous molar to the rst molar region. There were radiopaque tiny
ecks at the upper side of the lesion.

In December 2008, we performed enucleation of the mandibu-


lar tumor under general anesthesia. We also performed a Neumann
incision from the medial buccal aspect of the left mandibular
second deciduous molar to the alveolar crest, corresponding to
the distal buccal aspect of the rst molar. When we raised the
mucoperiosteal ap, we observed alveolar bone protrusion and par-
tial thinning. Following resection of the buccal alveolar bone, we
bluntly detached the tumor from the surrounding bone; we then
enucleated the tumor along with the hard tissue inside it en bloc,
and closed the wound with absorbable sutures. The uid in the
Fig. 2. CT images. (A) Sagittal image. A unilocular cystic radiolucent area was
tumor was yellowish-brown and serous. The tumor was detached observed from the left mandibular second deciduous molar periapical region to the
easily from the impacted left mandibular second premolar and rst distal root. At the upper side of this area, there was a tooth-like radiopaque area. (B)
molar, which were adjacent to the tumor, and demonstrated par- Axial image. Buccolingual jawbone protrusion, cortical bone resorption, and cortical
tial exposure of the tooth germ in the cavity after enucleation of bone thinning occurred. The mandibular canal is compressed and deviated at the
inferior border of the mandible by the tumor.
the tumor, as well as from the distal root of the left mandibular
second deciduous molar and the surrounding bone surface, which
were adjacent to the tumor. No adhesion was observed. Therefore, structure. The cyst wall-like tissue encapsulating this odontoma
rather than removing the left mandibular second premolar, rst demonstrated an odontogenic proliferative epithelium lining the
molar, and second deciduous molar, we chose to retain them. Cur- inner surface. The lining epithelium, composed of cuboidal and stel-
rently, at ve years after surgery, the deviated left mandibular rst late reticulum cells, contained ghost cells, which were eosinophilic
molar has erupted to the normal location, and the left mandibular and swollen. The nuclei of the ghost cells disappeared, leaving
second molar has demonstrated movement in a normal direction.
In addition, the distal root of the left mandibular second deciduous
molar, and the distal root that had demonstrated non-physiological
resorption due to the lesion were being lost following the pro-
gression of physiological resorption; the permanent left second
premolar has erupted to the normal location. We have not per-
formed occlusal guidance for the left mandibular second premolar,
rst molar, or second molar. The patient is now 10 years old, with
no apparent recurrence, and has made favorable progress (Fig. 3).
The resected material comprised hard tissue demonstrating a cystic
structure 20 mm 20 mm in size; in the lumen of the cyst wall-
like structure, hard tissue with an irregular surface was observed
Fig. 3. Panoramic radiograph 5 years after surgery. The tumor cavity disappeared;
(Fig. 4). The hard tissue, in which a cyst wall-like structure was
the left mandibular second premolar and rst molar, which had deviated due to the
observed, was histologically an odontoma composed of dentin with tumor, erupted to their normal locations; the left mandibular second molar moved
a tubular structure, an enamel matrix structure, and a cementum in a normal direction.
N. Moritani et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 421425 423

Generally, tumor extirpation is considered to be the primary


treatment. However, since the classication of CCOT as a cyst in
2005, fenestration has been performed in several cases, but also
was reportedly not effective in many cases [21]. Sato et al. [17]
reported that in seven of eight cases in which fenestration was per-
formed, the lesion did not subsequently demonstrate a tendency
toward reduction, and extirpation was subsequently performed;
they further stated that fenestration is not an appropriate treat-
ment for CCOT. In regard to histopathologic ndings in cases in
which resection was performed following fenestration, Chiba et al.
[22] reported that the original form of the CCOT lesion persisted
with little change, even at six months after fenestration; there-
fore, they stated that fenestration cannot be expected to reduce
the tumor cavity. Of the cases of CCOT in children aged <10 years
in Japan, fenestration was performed in three; in all of these, fen-
estration was followed shortly afterward by extirpation [7,17,18].
Rather than fenestration, tumor extirpation is considered to be the
appropriate rst-line treatment for CCOT [9,17].
Among cases of CCOT in children aged <10 years, a complete
impacted tooth was present in the tumor in four cases [1,7,15,16];
Fig. 4. Cross-sectional description of the extirpated specimen. The size of the extir- in three of these [7,15,16], the impacted tooth was removed when
pated specimen was 20 mm 20 mm. The surface was dark red, cystic soft tissue. the tumor was extirpated. In the other case [1], the tooth was pre-
Spherical hard tissue with an irregular surface was attached to the interior surface served; however, due to a poor prognosis, the tooth was scheduled
of the cyst wall.
to be extracted. Based on the above reports, extraction of impacted

behind only a pale outline. In addition, conglomerates of ghost cells


were also observed in the epithelium (Fig. 5). Based on the above,
we made a histopathologic diagnosis of CCOT with odontoma.

3. Discussion

Calcifying cystic odontogenic tumors were rst reported in 1963


by Gorlin et al. [2] under the name of calcifying odontogenic cysts.
Histopathologically, CCOT is a relatively rare disease characterized
by the appearance of ghost cells and calcication in the lining
epithelium derived from odontogenic epithelium [2,3]. Despite
their neoplastic character, they had been classied as a cystic
disease. However, in the 2005 revision of the World Health Organi-
zation Histological Classication of Odontogenic Tumors, the name
was changed to represent their neoplastic character. In addition, the
tumors were classied to show whether they were solid or cystic
[4,5]; cystic tumors were named CCOT, while solid tumors were
named dentinogenic ghost cell tumors [5].
Calcifying cystic odontogenic tumors are observed in all age
groups. Although they are considered to occur most frequently in
individuals in their teens, CCOT in children aged <10 years is rare
[6]. Including our own case, we found nine reports of CCOT in chil-
dren aged <10 years, among all Japanese reports from 1974 to 2014
(Table 1). The report by Murakami et al. [1] of CCOT in a 4-year-old
child is the only prior report we could nd of CCOT occurring in the
deciduous dentition period, thus making our case extremely rare.
On radiography, CCOT generally appears as a well-dened,
unilocular cystic radiolucent area; odontoma-like radiolucent
areas, impacted teeth, and unerupted teeth are also common [79].
However, there are also reports of CCOT presenting on radiography
with only a cystic radiolucent area [10], as well as multilocu-
lar areas [1113]; as these reports demonstrate, CCOT is diverse
[14]. All nine cases (including our own) of CCOT in children aged
<10 years [1,6,7,9,1518] were unilocular, while eight cases also Fig. 5. Histopathologic ndings (H&E staining, decalcied specimen, A: original
presented with odontoma. These cases included both radiolucent magnication, 10, B: original magnication, 100). (A) The hard tissue encap-
and radiopaque areas. Diseases associated with impacted teeth sulated by cyst wall-like tissue was composed of dentin with a tubular structure,
that should be differentiated from CCOT include: (1) adenoma- an enamel matrix structure, and a cementum structure. (B) The cystic structure was
lined with odontogenic epithelium, and cuboidal cells were arranged in the basal
toid odontogenic tumor, (2) ameloblastic broma, (3) ameloblastic
lamina. The lining epithelium revealed stellate reticulum and ghost cells (arrows),
odontoma, (4) ameloblastic bro-odontoma, (5) calcifying epithe- which were eosinophilic and swollen. The nuclei of the ghost cells disappeared,
lial odontogenic tumor, and (6) odontoma [19,20]. leaving behind only a pale outline.
424
Table 1
Reported cases of CCOT in patients under 10 years old in Japan (19742014).

Case Age/sex Region Appearance With or Impacted tooth Relationship Deviation of Root resorption of Diagnosis Treatment Treatment of Prognosis Author (y)
without inside the between lesion impacted tooth approximal tooth impacted tooth (follow-up
odontoma lesion and impacted caused by lesion in contact with associated period)

N. Moritani et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 421425
tooth lesion with lesion

1 9/F Right maxilla Unilocular With Supernumerary Inside lesion Not mentioned Not mentioned Dentigerous Extirpation Extraction of Not Eda et al.
tooth-like cyst supernumer- recorded (1974) [15]
deciduous ary
molar tooth
2 9/M Left mandible Unilocular With Left Inside lesion None None Dentigerous Extirpation Extraction of Good (3 y 2 Igari et al.
mandibular cyst left m) (1982) [16]
canine mandibular
canine
3 9/F Left maxilla Unilocular With Left maxillary Outside lesion Left maxillary None Maxillary cyst Extirpation Extraction of Good (1 y 8 Sato et al.
second molar rst molar after left maxillary m) (1982) [17]
fenestration rst and
second molar
4 9/F Right Unilocular With None Outside lesion Left mandibular Left mandibular Maxillary cyst Extirpation Extraction of Good (6 y 3 Misaki
mandible-left lateral deciduous lateral or odontogenic left m) et al.
mandible incisor-second incisor, deciduous tumor mandibular (1986) [9]
premolar, right second molar lateral incisor
mandibular
canine-rst
premolar
5 7/F Right Unilocular Without Right Inside lesion Right mandibular Not mentioned Dentigerous Extirpation Extraction of Good (1 y Fujisawa
mandible mandibular canine, second cyst after right 11 m) et al.
rst premolar premolar fenestration mandibular (1991) [7]
rst premolar
6 4/F Right maxilla Unilocular With Deciduous Outside lesion None None Compound Extirpation Conservation Good (7 m) Murakami
maxillary right odontoma et al.
incisor (1991) [1]
7 7/M Right maxilla Unilocular With None Outside lesion Right maxillary None Maxillary cyst Extirpation Conservation Good (1 y 9 Eda et al.
canine, second after m) (2003) [18]
molar fenestration
8 6/M Right maxilla Unilocular With None Outside lesion Right mandibular None Compound Extirpation Conservation Good (9 m) Hayashi
canine, rst odontoma or et al.
premolar dentigerous (2004) [6]
cyst with
compound
odontoma
9 5/M Left mandible Unilocular With None Outside lesion Left mandibular Left mandibular Left Extirpation Conservation Good (5 y) Present
second premolar, second molar mandibular case
rst molar tumor
N. Moritani et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 28 (2016) 421425 425

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None of the authors have any conict of interest to declare.
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