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Calcifying odontogenic cyst (COC) is an uncommon benign cystic neoplasm of odontogenic origin, which shows extensive diversity in its clinico-pathological appearances and biological behavior. It most commonly occurs in broader age group of 1-82 years in anterior partof jaw which is classically described by presence of ghost cells. There are variants of COC according to clinical, histopathological, and radiological characteristics.Therefore a proper categorization of the cases is needed for better understanding of the pathogenesis of eachvariant. Here we report a rare case of ameloblastomatouscalcifying ghost cell odontogenic cyst in 24 year old female with brief review of literature. Presence of ameloblastomatous proliferation and impacted caninepresented a diagnostic dilemma and was diagnosed bycareful radiographic and histopathogical interpretation. Long follow up and more case report are required to shed light on its behaviour as there scarcity of data of this lesion in literature.
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Ameloblastomatous calcifying ghost cell odontogenic cyst - a rare variant of a rare entity
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Ameloblastomatous calcifying ghost cell odontogenic cyst - a rare variant of a rare entity
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                                                                                                          Case report


Ameloblastomatous calcifying ghost cell
odontogenic cyst - a rare variant of a rare entity




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                                                                                         na
Harkanwal Preet Singh, MDS1                                  Introduction
Madhulika Yadav, MDS2




                                                                                      io
Amit Nayar, MDS1                                             Epithelial-lined cysts seldom occur in skeletal bones,
Chanchal Verma, MDS3                                         because embryonic epithelial rests are normally not
Palak Aggarwal, MDS3                                         found in them. They do, however, occur in the jaws
Sandeep Kumar Bains, MDS4                                    where the majorities are lined by epithelium derived from




                                                                                   az
                                                             remnants of the odontogenic apparatus. These odonto-
                                                             genic cysts are classified as either of developmental or
1
    Department of Oral and Maxillofacial Pathology and       inflammatory origin. The calcifying ghost cell odonto-
    Microbiology, Swami Devi Dyal Hospital and Dental        genic cyst (CGCOC) is a rare example of a developmen-




                                                                     n
    College, Barwala, Panchkula, Haryana. India              tal odontogenic cyst, its occurrence constituting about
2
    Department of Pedodontics and Preventive Dentistry,      0.37% to 2.1% of all odontogenic tumors (1). The calci-




                                                                  er
    Faculty of Dental Sciences, King Georgeʼs Medical        fying ghost cell odontogenic cyst (CGCOC) was first de-
    University, Lucknow, Uttar Pradesh, India                scribed by Gorlin et al. who were impressed by the sig-
3
    Department of Oral and Maxillofacial Pathology           nificant presence of the so-called ghost cells (2). At that


4
    Uttar Pradesh, India
                                         nt
    and Microbiology, ITS Dental College, Ghaziabad,

    Department of Oral Medicine and Radiology, Swami
    Devi Dyal Hospital and Dental College, Barwala,
                                                             time, they suggested that this cyst may represent the oral
                                                             counter part of the dermal calcifying epithelioma of Mal-
                                                             herbe (3, 4). Over the years since its first description, it
                                                             has become clear that the calcifying ghost cell odonto-
                                       iI
    Panchkula, Haryana. India                                genic cyst (CGCOC) has a number of variants, including
                                                             features of a benign odontogenic tumor. It was classified
                                                             as SNOMED code 9301/0, in the World Health Organi-
                                     on

Corresponding author:                                        zationʼs (WHO) publication Histological Typing of Odon-
Dr. Harkanwal Preet Singh, MDS                               togenic Tumors (5). WHO in 2005 have classified all
Oral and Maxillofacial Pathology and Microbiology,           COCs under the category of odontogenic tumours (6).
Swami Devi Dyal Hospital and Dental College,                 One of the histological features of the condition is the
                              i


Barwala, Panchkula, Haryana, India                           presence of ghost cells. Fejeskov and Krough are of the
                           iz


Phone: +91 8570023088                                        opinion that the lesion initially presents as a solid tumor,
E-mail: hkps0320@gmail.com                                   consisting mainly of ghost cells and that the cyst devel-
                                                             opment is a secondary phenomenon. They suggested a
              Ed




                                                             new descriptive term of ghost cell odontogenic tumor for
                                                             the lesion (7). We hereby, present a rare case report of
Summary                                                      ameloblastomatous calcifying ghost cell odontogenic
Calcifying odontogenic cyst (COC) is an uncommon be-         cyst in 24 year old female.
nign cystic neoplasm of odontogenic origin, which
shows extensive diversity in its clinico-pathological ap-
 IC




pearances and biological behavior. It most commonly oc-      Case report
curs in broader age group of 1-82 years in anterior part
of jaws which is classically described by presence of        24 year old female patient reported with the chief com-
ghost cells. There are variants of COC according to clin-
                                                             plaint of swelling on left side of face since 6 months. Pa-
C




ical, histopathological, and radiological characteristics.
                                                             tient was apparently asymptomatic 6 months back then
Therefore a proper categorization of the cases is needed
                                                             she noticed a pea sized swelling intra-orally in buccal
for better understanding of the pathogenesis of each
                                                             vestibule in 33, 34 region. Swelling gradually increased in
variant. Here we report a rare case of ameloblastomatous
                                                             size and manifested extra-orally on face. No associated
©




calcifying ghost cell odontogenic cyst in 24 year old fe-
male with brief review of literature. Presence of            pain or paraesthesia was present.
ameloblastomatous proliferation and impacted canine          General physical examination revealed that the patient
presented a diagnostic dilemma and was diagnosed by          was moderately built and nourished. All the vital signs
careful radiographic and histopathogical interpretation.     were within normal limits. Extra-oral examination re-
Long follow up and more case report are required to          vealed a firm swelling with normal coloured overlying skin
shed light on its behaviour as there scarcity of data of     on the left lower third of the face extending from corner
this lesion in literature.                                   of mouth to line joining outer can thus of eye to inferior
                                                             border of mandible (Fig. 1). None of the lymph nodes
Key words: cyst, ghost cells, neoplasm, odontogenic.         were palpable.

156                                                                            Annali di Stomatologia 2013; IV (1): 156-160
Ameloblastomatous calcifying ghost cell odontogenic cyst - a rare variant of a rare entity




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                                                                                            →
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                                                                      Figure 3. Orthopantomograph demonstrating a solitary well de-
                                                                      fined radiolucency (large red arrow) with sclerotic margins extend-
                                                                      ing from 38 to distal aspect of 45 and impacted 33 (small red ar-




                                                                                                az
                                                                      row).


                                                                      gion with buccal and lingual cortical plate expansion (Fig.
                                                                      4). Axial Computed tomography (CT) scan showed expan-




                                                                               n
                                                                      sion of the tumour mass with thinning of cortical outline
                                                                      (Fig. 5). 3-Dimensional computed tomography volumetric




                                                                            er
                                                                      reconstruction showed gross bone destruction with specks
                                                                      of calcification in mandibular anterior region (Fig. 6).




                                               nt
Figure 1. Extra-oral photograph showing a swelling extending
from corner of mouth to line joining outer can thus of eye to infe-
                                             iI
rior border of mandible.


Intra-oral examination revealed solitary well defined pain-
                                           on

                                                                                                  ➡
less swelling measuring 5 cm×3 cm, ovoid in shape, firm                                           ➡
                                                                                          ➡

in consistency, smooth texture with intact overlying mu-
cosa (Fig. 2).
                                                                                                           →
                                                                                                   ➡
                                  i
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               Ed




                                                                      Figure 4. Mandibular occlusal radiograph demonstrating well
                                                                      defined radiolucency (large red arrow) in mandibular anterior re-
                                                                      gion and impacted canine (small red arrow).
                                                                                           ➡
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                                                                                                                 →→
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Figure 2. Intra-oral photograph showing solitary well defined
swelling (large black arrow) measuring 5 cm × 3 cm, ovoid in
©




shape with intact overlying mucosa.


In roentenographic examination orthopantomograph
showed a solitary well defined radiolucency with sclerotic
margins extending from 38 to distal aspect of 45. Superi-
orly it extends up to alveolar process and inferiorly to 2 mm         Figure 5. Axial Computed tomography (CT) scan showing expan-
above the inferior border of mandible. It also showed im-             sion of the tumour mass to adjacent soft tissue at the buccal side
                                                                      on soft tissue shadow imaging and expansion (large red arrow),
pacted 33 (Fig. 3). Mandibular occlusal radiograph re-                thinning of cortical outline (small red arrow) with large unilocular
veals well defined radiolucency in mandibular anterior re-            radiolucency.


Annali di Stomatologia 2013; IV (1): 156-160                                                                                         157
H. P. Singh et al.




                                                                                                            → →
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                                 →
                         →




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Figure 6. 3-Dimensional CT volumetric reconstruction demon-           Figure 8. Photomicrograph showing ameloblastic islands(large
strated gross bone destruction (large red arrow) with specks of       black arrow) with stellate reticulum like areas (blue arrow) along
calcification (small red arrow) in mandibular anterior region.        with numerous ghost cells (small arrow) (H&E X10).




                                                                               n
Haematological and urine examinations did not reveal any




                                                                            er
abnormal findings. Clinical differential diagnosis of
dentigerous cyst, ameloblastoma and Calcifying cystic ep-
ithelial odontogenic tumour were given.
Incisional biopsy was performed and the tissue was sub-

                                               nt
mitted to the Department of Oral Pathology. Histopatho-
logic examination showed 6-8 layered cystic lining com-
posed of prominent basal cell layer with few areas of
                                             iI
palisading arrangement. Cells above the basal cell layer
are loosely arranged resembling stellate reticulum (Fig. 7).
                                  i        on
                               iz


                                                                      Figure 9. Photomicrograph showing dentinoid (red) and ghost
                                                                      cells (yellow) (Massonʼs trichrome; x10).
               Ed




                                                                      Based on these clinical, radiographic and histopatholog-
                                                                      ical features confirmatory diagnosis of Ameloblastoma-
                                                                      tous Calcifying odontogenic cyst was arrived. The oper-
                                                                      ation was performed under general anaesthesia by
                                                                      enucleation of the lesion and enucleated specimen was
                                                                      cystic approximately 5 mm to 6 mm in diameter, entire
 IC




                                                                      specimen was sent for histopathological evaluation, and
                                                                      it was reconfirmed as Ameloblastomatous calcifying ghost
Figure 7. Photomicrograph showing proliferating cystic lining
(large balck arrow) with stellate reticulum like areas. (H&E X10)     cell odontogenic cyst. The patient was followed up for 2
inset showing spherical to irregular calcification (small balck ar-   years with no evidence of recurrence.
C




rows) (H&E X10).

                                                                      Discussion
Numerous ameloblastic islands along with ghost cells
©




with distinct cell outline and numerous spherical calcifica-          Gorlin and colleagues identified CGCOC as a distinct
tions are seen within the epithelium and connective tissue            pathological entity in 1962 although according to Altini and
capsule (Fig. 8). Connective tissue is fibrocellular in na-           Farman, the condition had previously been described in
ture with chronic inflammatory cells, odontogenic islands,            German literature in 1932 by Rywkind (8). The CGCOC
spherical calcifications, few sebaceous nests and few                 has also been reported under a variety of other designa-
ghost cells with few multinucleated giant cells. Extensive            tions (Tab. 1).
areas of haemorrhage are also evident. Van gieson and                 In 1992, the World Health Organization (WHO) classified
massonʼs trichrome staining was done to differentiate                 CGOC as a neoplasm rather than a cyst but confirmed
ghost cell from other acidophilic bodies (Fig. 9).                    most of the cases are non-neoplastic. In view of this du-

158                                                                                      Annali di Stomatologia 2013; IV (1): 156-160
Ameloblastomatous calcifying ghost cell odontogenic cyst - a rare variant of a rare entity


Table 1. Terminology of the so-called calcifying odontogenic cyst (9). (Modified from Toida 2008)

Gorlin et al.                 1962             Calcifying odontogenic cyst

Gold                          1963             Keratinizing calcifying odontogenic cyst (KCOC)




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Bhaskar                       1965             Keratinizing ameloblastoma




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Fejerskov and Krogh           1972             Calcifying ghost cell odontogenic tumor (CGCOT)

Freedman et al.               1975             Cystic calcifying odontogenic tumor (COCT)




                                                                                               io
Praetorius et al.             1981             Dentinogenic ghost cell tumor (DGCT)

Ellis and Shmookler           1986             Epithelial odontogenic ghost cell tumor (EOGCT)




                                                                                            az
Colmenero et al.              1990             Odontogenic ghost cell tumor (OGCT)




                                                                             n
ality, many different terminologies have been applied to            opacities near apices of teeth. Presence of ghost cells
cystic and solid CGOC variants, but calcifying odonto-              are considered as characteristic but not pathognomonic




                                                                          er
genic cyst is the preferred term (9). Several classifications       for diagnosis of Calcifying odontogenic cyst. Numerous
of CGOC subtypes have been proposed, but most of                    ghost cells were observed in our case and many of
them have limitations in separating cystic and neoplastic           them have undergone calcification.
variant.

- Type 1. Cystic type:
     - (A) simple unicystic type,
                                             nt
First classification is proposed by Praetorius et al. (3):
                                                                    Ghost cells may undergo calcification and lose their cel-
                                                                    lular outline to form sheet like-area. Levy also supported
                                                                    the concept of calcification of ghost cells and held is-
                                                                    chemia as a responsible factor. Many investigators have
                                           iI
     - (B) odontoma-producing type,                                 made effort to clarify the nature of ghost cells by employ-
     - (C) ameloblastomatous proliferating type.                    ing special histochemical methods, transmission elec-
- Type 2. Neoplastic type: dentinogenic ghost cell                  tron microscopy, and scanning electron microscopy, and
                                         on

     tumor.                                                         various theories have been proposed without any general
The odontogenic origin of the CGCOC is widely ac-                   agreement. Gorlin et al., Ebling and Wagner, Gold,
cepted (10, 11). The cells responsible for the calcifying           Bhasker, Komiya et al., and Regezi et al. all believed that
odontogenic cyst are dental lamina rests (rests of Ser-             ghost cells represent normal or abnormal keratinization.
                                i


res) within either the soft tissue or bone. Therefore, cal-         Sedano and Pindborg thought the ghost cells represented
                             iz


cifying ghost cell odontogenic cysts are cysts of pri-              different stages of normal and aberrant keratin formation
mordial origin and are not associated with the crown of             and that they were derived from the metaplastic transfor-
an impacted tooth (12). It most often occurs as a central           mation of odontogenic epithelium. Other investigators
                Ed




(intraosseous) lesion (11, 12), whereas peripheral (ex-             suggested or implied that ghost cells may represent the
traosseous) localization in the soft tissue is rare (13, 14).       product of abortive enamel matrix in odontogenic epithe-
It occurs most commonly in 1 yr – 82 yrs of age. There              lium. However, the morphology of ghost cells seems dif-
is a distinct peak in the second decade. Equal sex                  ferent from that of enamel matrix. Ghost cells are not
predilection has been observed. Majority of the cases               unique to CGCOC, but are also seen in odontoma,
are seen in the jaw bones with equal distribution in                ameloblastoma, craniopharyngioma, and other odonto-
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maxilla and mandible. Anterior part of the jaws is the              genic tumors (8). Our case represents the classical fea-
commonest site of occurrence (15). In our present case              tures of calcifying odontogenic cyst, according to Praeto-
reported COC occurred in 24 year old female patient in              rius et al. (3) it comes under category of Type 1(c)
anterior region of mandible which falls well within the             ameloblastomatous proliferating type, and according to
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data given in the literature. According to Shear et al. sev-        Reichart (6), it comes under the category of calcifying
eral cases may cross the midline in the mandible, but it            ghost cell odontogenic cyst (CGCOC) non-neoplastic
is unusual in maxilla (4). In our case lesion extends from          (simple cystic) variant with unicystic, plexiform arneloblas-
38 and crossed the midline to reach the distal aspect of            tornatous proliferation of epithelial lining. Simple enucle-
©




45. This is also consistent with the findings observed in           ation is sufficient treatment, and there is little risk of recur-
literature. Radiographically, they appear as radiolucent            rence.
areas often associated with complex odontome leading                Ameloblastomatous COC microscopically resembles uni-
to the appearance of dense opacities in the cyst. Re-               cystic ameloblastoma except for the ghost cells and calci-
sorption of roots of adjacent teeth is a frequent finding           fications within the proliferative epithelium. Ameloblastom-
and a very important x-ray feature (15). Present case               atous COC occurs only intraosseously. This subtype of
showed well defined radiolucency extending from 38 to               COC is distinct from true ameloblastoma arising in COC.
45 with root resorption of 31, 32, 33, 41, 42, 44, 45 when          In contrast to ameloblastoma ex COC, the ghost cells and
radiograph was carefully evaluated it showed specks of              dystrophic calcifications are within the proliferative epithe-

Annali di Stomatologia 2013; IV (1): 156-160                                                                                    159
H. P. Singh et al.


lium, which lacks histopathologic criteria as suggested by                    plastic potential. Acta Odontologica Scandinavica 1981;
Vickers and Gorlin, and is confined to the cyst lumen.                        39:227-40.
Ameloblastoma ex COC designates an ameloblastoma                        4.    Shear M, Speight P. Calcifying odontogenic cyst (Calcifying
arising from the cyst lining epithelium of COC. Our review                    cystic odontogenic tumors). Cyst of the Oral and Maxillofa-
                                                                              cial Region, Blackwell Munksgaard, New York, NY, USA, 4th
of the literature revealed only four cases (16).
                                                                              edition, 2007; 100-107.




                                                                                                          li
Ameloblastoma ex COC occurs intraosseously, appear-                     5.    Kramer IRH, Pindborg JJ, Shear M. Histological typing of
ing as cyst-like, radiolucent lesions. Whether these tumors                   odontogenic tumours. 2nd ed, Springer-Verlag, Berlin 1992;




                                                                                                       na
are potentially as destructive as typical ameloblastoma                       7-20.
and have the same propensity for recurrence is unknown.                 6.    Philipsen HP. Keratocystic odontogenic tumour. In: Barnes
Whether ameloblastoma ex COC should be classified                             L, Eveson JW, Reichart P, Sidransky D (EDS). Head and
as a subtype of ameloblastoma or as a subtype of COC                          neck tumours. Pathology and Genetics. WHO Classification
                                                                              of tumours. IARC Press, Lyon, 2005: 306-7.




                                                                                                    io
may be open to discussion. Buchner suggested that if the
COC was associated with an ameloblastoma, its behav-                    7.    Fejerskov O, Krough J. The calcifying ghost cell odontogenic
                                                                              tumor or the calcifying odontogenic cyst. J Oral Pathology
ior and prognosis would be of the same as an ameloblas-
                                                                              1972; 1:273.
toma, not COC (16).




                                                                                                 az
                                                                        8.    Sonone A, Sabane VS, Desai R. Calcifying Ghost Cell
                                                                              Odontogenic Cyst: Report of a Case and Review of Litera-
                                                                              ture. Case Reports in Dentistry 2011; 1-5.
Conclusion                                                              9.    Toida M. So-called calcifying odontogenic cyst: review and
                                                                              discussion on the terminology and classification. J Oral




                                                                                  n
Calcifying odontogenic cyst has long been recognized as                       Pathol Med 1998; 27(2):49-52.
a distinct clinical entity. Some authorities believe that it is         10.   Hong SP, Ellis GL, Hartman KS. “Calcifying odontogenic




                                                                               er
more appropriately called as tumour (Dentinogenic ghost                       cyst. A review of ninety-two cases with reevaluation of their
cell tumour). It can sometimes pose a diagnostic dilemma                      nature as cysts or neoplasms, the nature of ghost cells, and
which should be diagnosed based upon vigilant compre-                         subclassification,” Oral Surgery Oral Medicine and Oral
hensive analysis of radiographic and histopathologic find-                    Pathology, vol. 72, no. 1, 1991; 56-64.


                                                 nt
ings. More number of case reports is required to exactly
delineate the biological behaviour of such a rare histolog-
ical variant of rare odontogenic cyst.
                                                                        11.   Marx RE, Stern D. “Odontogenic and nonodontogenic
                                                                              cysts,” in Oral and Maxillofacial Pathology: A Rationale for
                                                                              Diagnosis and Treatment, Quientessence Publishing,
                                                                              Hanover Park, Ill, USA, 1st edition, 2003; 607.
                                               iI
                                                                        12.   Altini M, Farman AG. “The calcifying odontogenic cyst: eight
                                                                              new cases and a review of the literature”. Oral Surgery
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2.    Gorlin RG, Pindborg JJ, Clausen FP, Vickers RA. The cal-                odontogenic cyst - Gorlinʼs cyst - report of two cases. Coll
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      cifying odontogenic cyst - a possible analogue of the cuta-             Antropol 2000; 28(1):357-62.
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                Ed




      1962; 15(10):1235-43.                                             16.   Nosrati K, Seyedmajidi M. Ameloblastomatous Calcifying
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160                                                                                         Annali di Stomatologia 2013; IV (1): 156-160
