See discussions, stats, and author profiles for this publication at: https://www.researchgate.

net/publication/263371222

Calcifying odontogenic cyst of anterior maxilla with complex odontoma

Article · June 2014

DOI: 10.4103/0975-2870.135290

CITATIONS READS
7 1,575

4 authors, including:

Yadavalli Guruprasad Dinesh Singh

Government Dental College and Research Institute, Ballari,India 60 PUBLICATIONS 328 CITATIONS
112 PUBLICATIONS 590 CITATIONS
SEE PROFILE
SEE PROFILE

All content following this page was uploaded by Yadavalli Guruprasad on 25 June 2014.

The user has requested enhancement of the downloaded file.

 ISSN: 0975-2870

Medical Journal
of
Dr.DY Patil University
Official Publication of Dr. D Y Patil University (Deemed University)

Volume 7 / Issue 4 / July-August 2014

www.mjdrdypu.org
 Case Report

Calcifying odontogenic cyst of anterior maxilla

with complex odontoma
Yadavalli Guruprasad, Dinesh Singh Chauhan, Vinod Kumar1, Prahalad Hunsagi2
Departments of Oral and Maxillofacial Surgery, 1Pedodontics and Preventive Dentistry, and 2Oral and Maxillofacial Pathology, AME’S Dental College
Hospital and Research Centre, Raichur, Karnataka, India

ABSTRACT Access this article online

Calcifying odontogenic cyst (COC) is an unusual and unique
lesion with characteristics of a solid neoplasm and of a cyst. It
shows considerable amount of histopathological diversity, with
variable clinical behavior such as cystic, neoplastic and infiltrating
malignant behavior. It is a rare developmental odontogenic
cyst with notable presence of histopathological features, which
include a cystic lining demonstrating characteristic “Ghost”
epithelial cells with a propensity to calcify. Odontomes are
best known as hamartomatous benign tumors rather than true
neoplasms, arising from odontogenic tissues. Histologically,
they are classified as compound and complex variety. We report
a rare case of COC of anterior maxilla with complex odontome
in a 12-year-old female child.
Keywords: Calcifying odontogenic cyst, complex odontoma,
maxilla
Introduction
True bone cysts are often encountered in facial bones due
to the presence of embryonic epithelial rests in these bones.
A majority of them are remnants of odontogenic apparatus.
The calcifying odontogenic cyst (COC) is a developmental
odontogenic cyst and its occurrence constitutes about
0.3-0.8% of all odontogenic cysts. Calcifying odontogenic
cyst is a rare lesion of the jaws first described as a distinct
entity by Gorlin et al., in 1962.[1] The condition is also
referred as Gorlin’s cyst, keratinizing ameloblastoma or
melanotic ameloblastic odontoma. COC is often referred
as an asymptomatic slow growing swelling of the jaws. It
is a well-circumscribed, solid or cystic lesion derived from
odontogenic epithelium, which develops from reduced
enamel epithelium or remnants of odontogenic epithelium in
the follicle, gingival tissue or bone, but contains “ghost cells”
and spherical calcifications. It is considered a unique entity
Quick Response Code:
Website:
www.mjdrdypu.org
DOI:
10.4103/0975-2870.135290
with both cystic and neoplastic behavior.[2,3] Odontomes
are best known as hamartomatous benign tumors rather
than true neoplasms, arising from odontogenic tissues.
Histologically, they are classified as compound and complex
variety. Complex odontoma is a mass of disorganized
dental tissues. Clinically, they are classified as intraosseous
(central), peripheral (soft-tissue or extra-osseous) and
erupted odontomes. The central (intraosseous) odontomes
are common representing 51%, occurring in anterior maxilla
(compound odontoma) followed by mandibular molar
region (complex odontoma).[4] We report a rare case of COC
associated with complex odontoma in a 12-year-old female
child with emphasis on its clinical features, diagnosis and
surgical management.
Case Report
A 12-year-old female patient came with a chief complaint
of asymptomatic swelling in the left upper jaw since 6
months. The lesion had been slowly increasing in size
since it was first noticed. Extraorally the lesion was
present on the anterior maxilla around 3 cm × 3.5 cm
in size from the midline of the upper lip to the corner
of the mouth thus displacing the left ala of the nose
and obliterating the nasolabial fold. The lesion was
extending from the left central incisor to first premolar
on the same side intraorally and soft in consistency on

Address for correspondence:

Dr. Yadavalli Guruprasad, Department of Oral & Maxillofacial Surgery, AME’S Dental College Hospital & Research Centre, Raichur - 584 103,
Karnataka, India. E-mail: guru_omfs@yahoo.com

508 Medical Journal of Dr. D.Y. Patil University | July-August 2014 | Vol 7 | Issue 4
 Guruprasad, et al.: Calcifying odontogenic cyst of anterior maxilla with complex odontoma

palpation [Figures 1 and 2]. A panoramic radiograph the decalcified section showed haphazard arrangement
showed a well-circumscribed radiolucency in relation to of enamel, dentin, cementum and pulp confirming it as
left upper anterior teeth and canine tooth causing root complex odontoma. There were no inductive changes
resorption and displacing roots of incisors [Figure 3]. like presence of dentinoid like material in the connective
Maxillary occlusal radiograph showing well defined tissue capsule [Figures 9-11]. Post-operative follow-up
unilocular radiolucency in relation to left anterior was done for 1 year and no recurrence was observed.
and canine teeth along with the odontome [Figure 4].
Computed tomography scan coronal and axial section Discussion
revealed well-circumscribed radiolucency in relation to
The COC is known to involve mandible and maxilla
left anterior and canine teeth along with the odontome
with equal frequency. The age of occurrence of the cyst
[Figures 5 and 6]. Fine needle aspiration cytology of the
has been reported to vary from 3 years to 80 years with
lesion was performed, which yielded straw colored fluid
definite peaking in the second decade. [1,3,4] The cyst is
and a provisional diagnosis of dentigerous cyst was made.
usually asymptomatic unless secondarily infected. Some
Patient was posted for surgical exploration under general
cases were reported where the cyst concomitantly occurred
anesthesia and the lesion was enucleated using intraoral
with other odontogenic lesions.[2,4] Praetorius[3] described
vestibular approach and the entire lesion was excised
four types of conjunctional lesions with the cyst: Namely,
along with the odontome [Figures 7 and 8]. The cavity was
dentine producing ameloblastoma, odontoameloblastoma,
closed primarily after aggressive curettage. The excised
tissue was sent for histopathological examination, which
revealed presence of cystic space lined by odontogenic
epithelium with ghost cells suggestive of Type 1 COC and

Figure 2: Submental view photograph showing obvious swelling on the

left anterior maxillary region obliterating nasolabial fold and displacing
the left ala of the nose

Figure 1: Frontal view photograph showing slight obvious swelling on

the left anterior maxillary region obliterating nasolabial fold

Figure 3: Orthopantomogram showing unilocular radiolucent lesion Figure 4: Maxillary occlusal radiograph showing well defined unilocular
in relation to left maxillary anterior and canine teeth thus displacing radiolucency in relation to left anterior and canine teeth. Note the arrows
the involved teeth showing the odontome

Medical Journal of Dr. D.Y. Patil University | July-August 2014 | Vol 7 | Issue 4 509
 Guruprasad, et al.: Calcifying odontogenic cyst of anterior maxilla with complex odontoma

Figure 5: Graphical illustration showing the cyst arising from the odontome. Figure 6: Computed tomography scan coronal view showing well
Note the shaded area a and b showing the odontome and cystic lining defined unilocular radiolucency in relation to left anterior and canine
teeth along with the odontome

Figure 7: Computed tomography scan axial view showing well defined

unilocular radiolucency in relation to left anterior and canine teeth along Figure 8: Intraoperative photograph showing the exposed cystic lining
with the odontome using the vestibular incision

Figure 9: Excised specimen showing cystic lining along with the Figure 10: Histopathological picture showing cystic space lined by
odontome. Note the arrow showing the odontome odontogenic epithelium and ghost cells (H and E, ×400)

ameloblastic fibro-odontoma and complex odontoma. of COC, Type 1: Simple monocystic type with presence of
The presence of ghost cells characterizes the histological ghost cells with or without dentinoid calcified tissue, Type
appearance of the lesion. Histologically there are 3 types 2: Formation of calcified tissues in the lumen of the cyst

510 Medical Journal of Dr. D.Y. Patil University | July-August 2014 | Vol 7 | Issue 4
 Guruprasad, et al.: Calcifying odontogenic cyst of anterior maxilla with complex odontoma
Figure 11: Decalcified section of showing disorganized enamel, dentin
and cemental matrix confirming the diagnosis of complex odontoma
(H and E, ×400)
wall, Type 3: Ameloblast like proliferation in the connective
tissue and lumen of the cyst may be seen.
The calcifications, if present, will appear scattered
radiopaque flakes in the radiograph. The other conditions
which may simulate this radiographic appearance are
the Pindborg tumor, ameloblastic fibro-odontoma and
adenomatoid odontogenic tumor. COC may occur in
association with other odontogenic tumors, the most
common of these is the odontoma. Hirshberg et al . [4]
reveals 52 cases of COC associated with odontoma, analyzes
clinical and histological features and it was classifies as a
separate entity. The term “odonto COC” was suggested. The
anterior region of the jawbones was the most commonly
affected site in both cases, but the COC with odontome
presents a female predominance (2:1) with a mean age
of 16 years, most frequently occurring in the maxilla
(61.5%). Radiographically appears as a mixed radiolucent-
radiopaque lesion (80.5%). Microscopically the epithelial
components in COC with odontome were identical to
those described for simple COC, but the former present’s
tooth-like structures that appear to be an integral part of
the lesion.
Several possibilities are suggested regarding the
pathogenesis of COC with odontome. One possibility
is that the COC and the odontoma may represent
coincidental juxtaposition of COC and an odontoma,
because other odontogenic tumors like ameloblastoma
have been reported to be associated with COC.[5] Other
investigators suggest that the COC develops secondarily
from odontogenic epithelium that participates in the
formation of the odontoma. However, it also has been
suggested that the odontoma develops secondarily from
lining epithelium of the COC.[6]
Medical Journal of Dr. D.Y. Patil University | July-August 2014 | Vol 7 | Issue 4
The etiology of odontoma is not known but environmental
causes such as infection, trauma, family history and genetic
mutation are hypothesized.[7,8] The complex odontoma
constitute 5-30% of all odontogenic tumors and are mostly
found in the posterior mandible and anterior maxilla. They
are seen with unerupted teeth in 10-44% and about 17% of
them are associated with impacted maxillary lateral incisors.
Females are affected marginally more than male (1.5:1).
World Health Organization classified odontomes into three
groups: (a) complex odontome; when the calcified dental
tissues are simply arranged in an irregular mass bearing no
morphological similarity to rudimentary teeth. (b) Compound
odontoma: composed of all odontogenic tissues in an orderly
pattern, which result in many teeth-like structures, but
without morphological resemblance to normal teeth. (c)
Ameloblastic fibro-odontome: consists of varying amounts of
calcified dental tissue and dental papilla-like tissue, the later
component resembling an ameloblastic fibroma.[8]
The ameloblastic fibro-odontome is considered as an
immature precursor of complex odontome. Odontomes
are incidental findings on routine radiographs. Retention
of deciduous teeth, unerupted permanent teeth, cortical
expansion and teeth displacement are all indicators for
a possible odontomes. [9] They are symptomatic with
expansion, local and/or radiating pain and neurosensory
deficit when secondarily infected. [10] The differential
diagnosis of complex odontoma include cementifying or
ossifying fibroma, adenomatoid odontogenic tumor, COCs,
periapical cemental dysplasia and calcifying epithelial
odontogenic tumor.[11]
Conclusion
COC occurring along with complex odontoma is a rare
finding among the cystic lesions of the jaws. Early diagnosis
and management is important to prevent its potential
complications like involvement of the nasal floor and
maxillary sinus and progression to more aggressive tumor
masses thus causing severe morbidity.
References
1. Gorlin RJ, Pindborg JJ, Odont, Clausen FP, Vickers RA. The
calcifying odontogenic cyst — A possible analogue of the
cutaneous calcifying epithelioma of Malherbe. An analysis of
fifteen cases. Oral Surg Oral Med Oral Pathol 1962;15:1235-43.
2. Freedman PD, Lumerman H, Gee JK. Calcifying odontogenic
cyst. A review and analysis of seventy cases. Oral Surg Oral
Med Oral Pathol 1975;40:93-106.
3. Praetorius FP. Calcifying odontogenic cyst. Range, variation
and neoplastic potential. Symposium on maxillofacial bone
pathology. Int J Oral Surg 1975;4:89.
511
 Guruprasad, et al.: Calcifying odontogenic cyst of anterior maxilla with complex odontoma
4. Hirshberg A, Kaplan I, Buchner A. Calcifying odontogenic
cyst associated with odontoma: A possible separate entity
(odontocalcifying odontogenic cyst). J Oral Maxillofac Surg
1994;52:555-8.
5. Lello GE, Makek M. Calcifying odontogenic cyst. Int J Oral
Maxillofac Surg 1986;15:637-44.
6. Shamaskin RG, Svirsky JA, Kaugars GE. Intraosseous and
extraosseous calcifying odontogenic cyst (Gorlin cyst). J Oral
Maxillofac Surg 1989;47:562-5.
7. Toida M, Ishimaru J, Tatematsu N. Calcifying odontogenic cyst
associated with compound odontoma: Report of a case. J Oral
Maxillofac Surg 1990;48:77-81.
8. Philipsen HP, Reichart PA, Praetorius F. Mixed odontogenic
tumours and odontomas. Considerations on interrelationship.
Review of the literature and presentation of 134 new cases of
odontomas. Oral Oncol 1997;33:86-99.
512
View publication stats
9. Phillips MD, Closmann JJ, Baus MR, Torske KR, Williams
SB. Hybrid odontogenic tumor with features of ameloblastic
fibro-odontoma, calcifying odontogenic cyst, and adenomatoid
odontogenic tumor: A case report and review of the literature.
J Oral Maxillofac Surg 2010;68:470-4.
10. Oliveira JA, da Silva CJ, Costa IM, Loyola AM. Calcifying
odontogenic cyst in infancy: Report of case associated with
compound odontoma. ASDC J Dent Child 1995;62:70-3.
11. Ozeç I, Kiliç E, Yeler H, Göze F, Yeler D. Large complex
odontoma associated with a primary tooth. Quintessence Int
2007;38:521-4.
How to cite this article: Guruprasad Y, Chauhan DS, Kumar V,
Hunsagi P. Calcifying odontogenic cyst of anterior maxilla with
complex odontoma. Med J DY Patil Univ 2014;7:508-12.
Source of Support: Nil. Conflict of Interest: None declared.
Medical Journal of Dr. D.Y. Patil University | July-August 2014 | Vol 7 | Issue 4