Arch Iranian Med 2009; 12 (4): 417 – 420

Case Report

Ameloblastomatous Calcifying Odontogenic Cyst: A Case

Report of a Rare Histologic Variant
•
Kamran Nosrati DMD*, Maryam Seyedmajidi DMD **

Calcifying odontogenic cyst is an uncommon developmental odontogenic cyst first described

by Gorlin in 1962. It is considered as an extremely rare cyst and accounts for only 1% of jaw cysts
reported. Because of its diverse histopathology, there has always been confusion about its nature
as a cyst, neoplasm, or hamartoma. In this report, we present a rare case of calcifying odontogenic
cyst with ameloblastic proliferation—an extremely rare histologic variant in a 22-year-old male in
the right mandibular molar region. The lesion was surgically removed. After enucleation no
recurrence has been recorded in the ensuing 14 months.

Archives of Iranian Medicine, Volume 12, Number 4, 2009: 417 – 420.

Keywords: Ameloblastic proliferation • calcifying odontogenic cyst • ghost cell • Gorlin cyst

Introduction right molar region extending from the first to the

third molar. The mucosa overlying the lesion was

C
alcifying odontogenic cyst (COC) was
first categorized as a distinct entity by
Gorlin et al.,1 and was named after him
since then. According to Shear,2 it accounts for 1%
of jaw cysts. As the number of reports increased, it
was proposed that COC was indeed a
heterogeneous group of entities with distinct
histopathologic findings. In this report, we present
a case of ameloblastomatous COC.
Case Report
A 22-year-old male patient was referred to an
oral and maxillofacial surgeon in November 2005
with a painless swelling in the right molar region
of the mandible that had been present for
approximately 20 days. No unusual features were
noted in extraoral examination. Intraorally, there
was a firm enlargement in the buccal and lingual
intact. Detailed examination of the involved teeth
revealed no mobility or tenderness to palpation.
There were also signs of caries at the first molar.
There was nothing special in his medical history.
Panoramic radiography and computed tomography
revealed a well-defined unilocular radiolucent
lesion involving the posterior region of the
mandible, extending from the first to third molar.
The margins of the lesion were scalloped. The first
and second right molars exhibited mild root
resorption (Figure 1). The differential diagnosis
included unicystic ameloblastoma and odontogenic
keratocyst. The lesion was surgically removed, and
involved teeth were also removed. The first
histopathologic diagnosis reported by a general
pathologist was ameloblastoma. The patient's

Authors' affiliation: *Department of Oral and Maxillofacial

Surgery, **Department of Oral and Maxillofacial Pathology,
Dental Faculty, Babol University of Medical Sciences, Babol,
Mazandaran, Iran.
•Corresponding author and reprints: Maryam Seyedmajidi
DMD, Department of Oral and Maxillofacial Pathology, Dental
Figure 1. Panoramic radiography of the case
Faculty, Babol University of Medical Sciences, Babol,
Mazandaran, Iran. revealed a well-defined unilocular radiolucent lesion
Tel: +98-111-229-14-08-EXT 116 involving the posterior region of the mandible,
Fax: +98-111-229-10-93, E-mail: ms_majidi79@yahoo.com extending from the first to the third molars; the
Accepted for publication: 4 September 2008 margins of the lesion were scalloped.

Archives of Iranian Medicine, Volume 12, Number 4, July 2009 417


Figure 2. Ghost cells in the cystic epithelium and
juxtaepithelial hyalinization (H&E, ×100).
specimen was referred to an oral and maxillofacial
pathologist whose diagnosis of the same biopsy
was ameloblastomatous COC.
Microscopic examination revealed ghost cells
in the cystic epithelium and juxtaepithelial
hyalinization (Figure 2). Acanthomatous
ameloblastic islands were seen in the connective
tissue lining of the cyst (Figure 3). Basal cell
hyperchromatism, vacuolization, and nuclear
polarization, which are often seen in
ameloblastoma, were absent (Figure 4).
In a postsurgical follow-up of 14 months, the
patient did not manifest any recurrences (Figure 5).
Discussion
The COC is an uncommon lesion that
Figure 3. Acanthomatous ameloblastic proliferation in
the connective tissue and dentinoid (H&E, ×40).
418 Archives of Iranian Medicine, Volume 12, Number 4, July 2009
Ameloblastomatous calcifying odontogenic cyst
Figure 4. In ameloblastic proliferation, basal cell
hyperchromatism, vacuolization, and nuclear
polarization, which are often seen in ameloblastoma,
are absent (H&E, ×400).
demonstrates considerable histologic diversity and
presents variable clinical behaviors. Although, it is
widely considered to represent a cyst, some
investigators prefer to classify it as a neoplasm.
Some COCs appear to represent non-neoplastic
cysts; other members of this group, variously
designated as dentinogenic ghost cell tumors or
epithelial odontogenic ghost cell tumors, having no
cystic features, may be infiltrative or even
malignant, and are regarded as neoplasms.3
In addition, the COC may be associated with
other recognized odontogenic tumors, most
commonly odontomas. However, adenomatoid
odontogenic tumors and ameloblastoma have also
been associated with COCs. Treatment and
prognosis are likely to be the same as for the
associated tumors.3 The WHO classification of
odontogenic tumors considers the COC with all its
variants as an odontogenic tumor rather than an
odontogenic cyst, although it describes that further
experience may provide more reliable criteria for
classification of the variants.3
Praetorius et al., Hong et al., and Buchner4–6
have attempted to classify the COC based on the
dualistic concept in contrast to the earlier monistic
Figure 5. Panoramic radiography of the case after 14
months.

concept. However, it seems the question
concerning the nature of COC has been solved
more recently by Toida who classified COC into a
cyst and a neoplasm.7 The neoplasm is divided into
benign and malignant type. The calcifying ghost
cell odontogenic tumor (CGCOT)—is used for
benign neoplasm and it may appear to be either
cystic or solid in architecture. The cystic and solid
variant of CGCOT may be named ‘cystic CGCOT’
and ‘solid CGCOT’, respectively. Thus, the lesion
showing cystic architecture and an extensive
intramural ameloblastoma such as proliferation
may be classified as the cystic CGCOT.
Ameloblastomatous COC microscopically
resembles unicystic ameloblastoma except for the
ghost cells and calcifications within the
proliferative epithelium. Ameloblastomatous COC
occurs only intraosseously. This subtype of COC is
distinct from true ameloblastoma arising in COC.
In contrast to ameloblastoma ex COC, the ghost
cells and dystrophic calcifications are within the
proliferative epithelium, which lacks
histopathologic criteria as suggested by Vickers
and Gorlin,8 and is confined to the cyst lumen.
Ameloblastoma ex COC designates an
ameloblastoma arising from the cyst lining
epithelium of COC (Figure 5). Our review of the
literature revealed only four cases.5,9,10
Ameloblastoma ex COC occurs intraosseously,
appearing as cyst-like, radiolucent lesions.
Whether these tumors are potentially as destructive
as typical ameloblastoma and have the same
propensity for recurrence is unknown.
Whether ameloblastoma ex COC should be
classified as a subtype of ameloblastoma or as a
subtype of COC may be open to discussion.
Buchner6 suggested that if the COC was associated
with an ameloblastoma, its behavior and prognosis
would be of the same as an ameloblastoma, not
COC.
The classification advocated by Hong et al.5 has
two categories for COC associated with
ameloblastoma: the ameloblastomatous cystic
variant and the neoplastic variant associated with
ameloblastoma. The former is characterized by a
unicystic structure in which the lining epithelium
shows unifocal or multifocal intraluminal
proliferative activity that resembles
ameloblastoma, although it also contains isolated
or clustered ghost cells and calcifications. The
latter is called ameloblastoma arising from COC
(ameloblastoma ex COC). It is characterized
histopathologically as comprising few or no ghost
Archives of Iranian Medicine, Volume 12, Number 4, July 2009
K. Nosrati, M. Seyedmajidi
cells with calcifications observed in the
transformed ameloblastomatous epithelial portion,
while the cyst lining of the epithelium contains
considerable number of ghost cells and
calcifications.11
In the present case, although the basal cells
showed ameloblastic proliferative activity, they did
not completely meet the histopathologic criteria of
early ameloblastoma as suggested by Vickers and
Gorlin.4 Hence, the case has been diagnosed as
ameloblastomatous COC and it has been fit into
the category ‘cystic CGCOT’ as suggested by
Toida.7,12
At this present state, it is very difficult to
determine whether any individual lesion having a
cystic architecture is truly cystic or, in fact,
neoplastic in nature. An extensive and systematic
analysis of many more cases including
immunohistochemical investigations on cell
proliferation activity may help resolve this
problem.
Ameloblastomatous COC is a rare histologic
variant. We found only 13 cases of
ameloblastomatous COC in the literature. Our case
did not show any evidence of recurrence after
treatment, but there is no doubt that careful post-
operative observations are necessary for COCs
which are associated with an ameloblastoma.
References
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The Portal of National Garden, Tehran/Iran

(photo by Afshin Bakhtiya, Gooya House of Culture & Art)

420 Archives of Iranian Medicine, Volume 12, Number 4, July 2009