ISSN 2231-0746

Vol 2 / Issue 1 / Jan-Jun 2012

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Annals of Maxillofacial Surgery
Official Publication of The Indian Academy of Maxillofacial Surgery
www.amsjournal.com Estd : 1996
 Case Report - Cyst & Tumours

Calcifying odontogenic cyst with atypical features

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S. M. Balaji, Thavarajah Rooban1
www.amsjournal.com Balaji Dental and Craniofacial Hospital, Teynampet, Chennai,
DOI: 1
Quality Diagnostics, Alwarpet, Chennai, India
10.4103/2231-0746.95331
Quick Response Code:
Address for correspondence:
Prof. S. M. Balaji, Consultant Craniofacial Surgeon and Director,
Balaji Dental and Craniofacial Hospital, 30, KB Dasan Road, Teynampet, Chennai – 600 018, India.
E-mail: [email protected]

ABSTRACT

The calcifying odontogenic cyst (COC) was first delineated in 1962. 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. In addition, the COC may be associated with other recognized odontogenic tumors. This gives rise to
a spectrum of variants of COC according to clinical, histopathological, and radiological characteristics. Very few reports have
actually captured the actual transformation while most reported cases are documents of co-existing lesions. This article presents
one such entity, where the asymptomatic presentation misled the diagnosis and on histopathological examination revealed
the COC with areas suggestive of adenomatoid odontogenic tumor.

Keywords: Adenomatoid odontogenic tumor, calcifying odontogenic cyst, cysts, epithelial–mesenchymal interaction,
odontogenic neoplasm, periapical lesion

INTRODUCTION presentation of COC is not rare. It has been reported to occur in

association with odontogenic entities suggestive of adenomatoid
In clinical situations, unusual presentation of uncommon lesion or odontogenic tumor (AOT).[3]
early presentation of rare lesions can mimic routine pathological
entities. The signs and symptoms of such lesions are often The aim of this article is to present a case that was nonsymptomatic,
misleading and pose a diagnostic and therapeutic challenge. which initially mislead the diagnosis. It highlighted the
They may require an alternative in treatment plan which in turn fact that a common clinical diagnosis could be a rare and
could influence the outcome of treatment. Uncommonly, rare histopathological surprise. This article also discusses the clinical
pathologies mimic the commonly encountered typical periapical and histopathological correlation of this condition and its impact
lesions. on treatment/prognosis.

True bone cysts are often encountered in facial bones due to the CASE REPORT
presence of embryonic epithelial rests in these bones. A majority
of them are remnants of odontogenic apparatus. The calcifying A 43-year-old male electrician reported to our center for prosthetic
odontogenic cyst (COC) is a developmental odontogenic cyst replacement of his missing lower posterior teeth. His medical
and its occurrence constitute about 0.3–0.8% of all odontogenic history and dental history was non-contributory except that he
cysts.[1] Since its description in 1930's and delineation as a unique had multiple uneventful extractions of mandibular second and
entity in 1960s, owing to the variations it presents with respect to third molars as well as maxillary third molars several months back.
the histopathology, an extensive classification has been proposed On clinical examination, no abnormalities were observed, except
for this entity with four groups and several subgroups.[2] The for an indistinct, nontender, hard bulge over the right maxillary
documented literature confirms that that COC has a spectrum of canine-lateral incisor region on palpation and also had attrition
variants, ranging from that of a developmental odontogenic cyst of anterior teeth. The right maxillary canine and lateral incisor
to benign and possibly a malignant odontogenic tumor.[2] Atypical appeared to be diverged. Few posterior teeth also had dental

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 Balaji and Rooban: Atypical calcifying odontogenic cyst

caries in various stages. Routine panoramic examination revealed of the periapical granuloma was planned after root canal
a radiolucent lesion in the right anterior region, measuring 1.7 treatment.
cm  1.4 cm [Figure 1]. The oval, radiolucent lesion involved
the distal part of the root of lateral incisor, the apex, and mesial On elevating a wide, semilunar flap, a well-circumscribed
part of the root of the right maxillary canine with diffuse margin. area of elevated, thinned cortex was identified [Figure 2]. On
Closer radiological examination with periapical radiography removal of the bone over the lesional area, an ovoid fluctuant,
revealed that the maxillary right canine root was in juxtaposition swelling with what clinically appeared to be a thick capsule
with the entity while the lateral incisor had a loss of lamina dura was identified. Carefully, the margins were explored and the
[Figure 1]. Clinically, there was a delayed response to pulp vitality entire lesion was removed in total after careful blunt dissection
test, indicating a compromise in pulpal vitality in both the teeth. [Figure 3]. The residual bone was clear with no evidence of cystic
lining [Figure 4A]. The entire area was thoroughly curetted till
healthy bone with pinpoint bleeding spots in bone appeared.
The patient had a parafunctional, occupation related habit
Apicectomy and retrograde filling was done in lateral incisor and
of traumatizing his canines repeatedly. Based on the history,
canine. The area was irrigated with Povidone-Iodine, saline, and
radiographic finding, clinical presentation, and pulpal vitality
primarily closed.
test a provisional diagnosis of periapical granuloma was
made. Clinical differential diagnosis of a lateral periodontal
The macroscopic appearance was nonspecific, except for the
cyst, squamous oontogenic tumor, and globulomaxillary cyst prominent superficial vasculature [Figure 4B and C]. The cystic
were considered. The nontender, indistinct, clinical bulge was mass was subjected to histopathological study. The lesion
attributed as a reactive, buccal cortical expansion of this probable exhibited cystic lumen lined by a thin layer of odontogenic
long-standing condition. Uneventful, single visit root canal epithelium (OE). The basal layer of this OE was made up of
treatment was done in canine and lateral incisors. The curettage

Figure 2: The lesional area after raising the semilunar flap. Note the shape
Figure 1: Orthopantomogram of the patient; asterix indicates the extent of the lesion and bone overlying the cyst
and nature of the radioluceny in between the apices of the right maxillary
canine and lateral incisor. Inset shows the periapical radiograph of the area

Figure 4: Composite image of A. The residual bone with healthy bleeding

after removal of the cyst B. Buccal surface of the cyst C. Lingual surface
Figure 3: Total removal of the cystic content in toto of the lesion

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 Balaji and Rooban: Atypical calcifying odontogenic cyst

palisaded columnar or cuboidal cells, resembling ameloblasts of should be taken to rule out periodontal cyst in such conditions.
varying thickness. The hyperchromatic nuclei of these cells were Electricians often engage in parafunctional oral habits such using
polarized away from the basement membrane. These cells were teeth to strip wires or hold screws. Minor, repeated such insults
overlined by loosely arranged epithelial cells resembling stellate could compromise the vitality of the pulp. In the present case, as
reticulum. There were a number of epithelial ghost cells which also observed in radiographs, the cuspal tips of maxillary canines
were devoid of nuclei, eosinophilic, and retaining their basic cell are blunt indicating the extent of parafunctional nature. Hence
outline. These eosinophilic ghost cells were often, enlarged, and clinically the presentation was thought to be that of a periapical
were of different shape with well-defined outlines. A few of them lesion. Absence of a well-defined sclerotic border in imaging
contained nuclear remnants [Figure 5]. indicated that the lesion did not evoke a chronic healing reaction
and probably the lesion is a periapical granuloma.
In one area of the section, thickening of OE was seen. In such
areas, islands of odontogenic cells were arranged in the form of The periapical curettage was planned. As the surgeon encountered
thin anastomosing strands in a plexiform and lattice-work pattern. a cystic swelling, after removal of bone over the lesional area,
Toward the center of the lesion, in this area, arrangement of the working diagnosis was proved wrong and an enucleation was
ameloblast-like cells in the form of ductal or rosette pattern was planned to remove the cyst in toto. The dense connective tissue
observed [Figure 6]. In the center of this ductal arrangement was a capsule facilitated the in toto removal of the lesion. The cystic
pale, eosiniophilic material with varying texture and pattern. Also lesion was carefully removed. As there was a strong attachment of
in one particular foci, cholesterol clefts were identified [Figure the capsule to bone, careful repeated curettage of residual bone
6B]. Based on the histopathological picture, a diagnosis of COC was essential to prevent recurrence. The area was closed after
with AOT-like areas was made. careful debridement. Healing was uneventful.

The healing was uneventful and patient was followed up for 6 The histopathological study threw more light into the composition
months with no evidence of recurrence. of the lesion. The lesion predominantly was COC with certain
areas suggestive of AOT. Similar cases have been reported in
DISCUSSION maxillofacial surgical literature.[3,5,6]

Periapical pathologies are common in oral and maxillofacial Odontogenic pathologies arise from odontogenic epithelium (OE).
surgical practice, and periapical cysts are more than 50% of all OE by itself has the potential for diverse differentiation under the
odontogenic cysts reported.[1] Asymptomatic periapical lesions influence of the ectomesenchyme. In this case, the proliferation of
are not uncommon and routine classical description of the strands of lesional tissue resembled the inner enamel epithelium
radiological appearance of the periapical cysts are a round or and hence possibly had the primary ectomesenchymal induction
ovoid radiolucency lined by a narrow radiopaque margin that potential. It has been pointed that the combined occurrence of
extends from the lamina dura of the involved tooth. In enlarging COC with odontogenic pathology is a possibility owing to the
cysts, this margin may be absent. A periapical cyst involving inherent potentiality of the OE. The cystic lesion could assume
proximal surface of tooth, with or without root resorption, is any part of the spectrum. Depending on its degree and direction
not uncommon and is attributed to accessory root canals.[4] Care of differentiation, the initial inductive stimulus, the degree of

Figure 5: A) The area showing thin OE with basal cells resembling Figure 6: A) Foci showing arrangement of solid areas of odontogenic
ameloblasts, ghost cells, and extravasated red blood cells. (H and E cells near cystic lumen with cords and lattice pattern, indicative of AOT
section, 20) B) A foci showing the thickened lining with palisaded basal features (H and E section, 4) B) Foci showing areas of cholesterol clefts
cells resembling ameloblasts, ghost cells, and extravasated red blood in an uninfected area. C) Foci showing features suggestive of AOT. Note
cells. (H and E section, 10) C) A foci showing thickened lining with the ductal or rosette-like appearance, reversal of polarity, centrally
palisaded basal cells resembling ameloblasts, ghost cells, superficial placed atypical material that has varying pattern (asterix), prominent
stellate reticulum-like cells, and extravasated red blood cells. (H and E intercellular bridges, and duplicated basal membrane in certain areas.
section, 40) (H and E section, 20)

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 Balaji and Rooban: Atypical calcifying odontogenic cyst

“odontogenesis” prior to the stimulus, and individual human treatment of COC is enucleation and the response depends on any
variability, the second lesion might have been initiated.[7] associated odontogenic tumor.[2] As AOT has good and favorable
prognosis, the condition would not recur again probably in future.
In the present case, the etipopathogenesis of COC could be However, we intend and continue to follow up the patient for
from either a mutated clone of presecretory ameloblast or a further monitoring and documentation.
postsecretory ameloblast that has acquired its mesenchymal
inducting potential. It is probably the earlier one that has CONCLUSION
served as the source of the neoplastic clone. In course of the
disease process, they have produced the characteristic ghost A case that had been initially misdiagnosed as periapical
cells. Probably, if left for more time, there could have been the pathology and was later identified as COC has been presented.
characteristic calcifications of the COC. The proliferating cells, at Clinician has to carefully assess all the features before treatment
some point of time, by their induction potential have produced planning. Intraoperative changes in findings have to be included
features similar to AOT in certain areas near the lumen. It is to for diagnosis and treatment modified in the better interest of the
be considered that in AOT, the duct-forming cells are reported to patient. The case probably is an apt example of a COC giving
possess secretory granules and coated vesicles near the luminal rise to an odontogenic pathology. Furthermore, this case stresses
pole, a feature which is highly reminiscent of preameloblasts. the fact that all enucleated specimen need to be examined
These structures were also reported in the non-duct forming histopathologically to confirm the working diagnosis, so as
columnar cells in various patterns and quantities.[8] Probably this appropriate precautious treatment plan and follow-up can be
case is the one of the few that serves as evidence to the hypothesis instituted.
that AOT could evolve from the pluripotent cell lining of COC.
If the current case had been diagnosed at a later stage, the lesion REFERENCES
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of red blood cells in the tissue section could be due to minor ninety-two cases with reevaluation of their nature as cysts or neoplasms,
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7. Zhang W, Chen Y, Geng N, Bao D, Yang M. A case report of a hybrid
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lesion. Although conventional imaging did not reveal any Oxford, UK: Blackwell Publishing Ltd.; 2007. p. 138.
calcification, literature cites that computed tomography had better 11. Sonone A, Sabne VS, Desai R. Calcifying Ghost cell oontogenic cyst:
Report of a case and review of literature. Case Rep Dent 2011;Article
accuracy in such situations.[9]
ID 328743, 5 pages, 2011. doi:10.1155/2011/328743.

The present case concurs with previous reported literature

Cite this article as: Balaji SM, Rooban T. Calcifying odontogenic cyst with
regarding COC. The case which occurred in a male in fifth decade atypical features. Ann Maxillofac Surg 2012;2:82-5.
of life, remained asymptomatic, presented as hard swelling. The
Source of Support: Nil, Conflict of Interest: None declared.
diagnosis was made on routine radiological examination.[11] The

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