Dentomaxillofacial Radiology (2004) 33, 152157

q 2004 The British Institute of Radiology

http://dmfr.birjournals.org

SYSTEMATIC REVIEW

Clinical and radiological features of odontogenic ghost cell

carcinoma: review of the literature and report of four new cases
Y Cheng, X Long*, X Li, Z Bian, X Chen and X Yang
Department of Oral and Maxillofacial Surgery, College of Stomatology, Wuhan University, Wuhan, Hubei, PR China 430079

Objectives: To analyse systematically the clinical and radiological features of odontogenic ghost
cell carcinoma (OGCC).
Methods: Clinical and radiological features of 22 OGCCs (4 new and 18 from the literature) were
analysed.
Results: There were 17 (77%) males and 5 (23%) females (male-to-female ratio of 3.4:1). Ages
ranged from 13 years to 72 years (mean 36.7) with a peak in the fourth (40.9%) and fifth (27.3%)
decades. The maxilla was involved in 68% and the mandible in 32%. Our study confirmed that
OGCC is more prevalent in Asians (12/18) than in other racial groups. The mixed radiolucent and
radiopaque lesion pattern was the most frequent (14/19) compared with radiolucent lesions (5/19).
89% (17/19) showed poorly defined borders and 11% (2/19) showed well defined borders. Root
resorption was reported in 31% (6/19) of patients and tooth displacements in 21%.
Conclusions: OGCC demonstrates clinical and radiographic features of a malignant tumour with
high recurrence.
Dentomaxillofacial Radiology (2004) 33, 152157. doi: 10.1259/dmfr/67909783
Keywords: calcifying; odontogenic; carcinoma; cyst; tumour; jaw

Introduction
The calcifying odontogenic cyst (COC), classified as an
odontogenic tumour by the World Health Organisation
(WHO), was first identified as a distinct entity by Gorlin
et al in 1962.1 In 1972, Fejerskov and Krogh2 used the label
calcifying ghost cell odontogenic tumour. In 1981,
Praetorius et al3 recognized four different histological
patterns of COC and classified them as type 1A (simple
unicystic), type 1B (odontome-producing), type 1C
(ameloblastomatous proliferating) and type 2 (dentinogenic ghost cell tumour). In their opinion, type 2 shares
many of the histological features of the cystic variants;
however, the solid growth pattern of the type 2 suggests
that its classification as a neoplasm is more appropriate.
Ellis and Shmookler4 used the term epithelial odontogenic
ghost cell tumour (EOGCT) for the neoplastic variant of
COC. Although the terminology is inconsistent, ghost cells
*Correspondence to: Xing Long Department of Oral and Maxillofacial Surgery,
College of Stomatology, Wuhan University, Wuhan, Hubei, PR China 430079;
E-mail: longxing_china@hotmail.com
Received 28 October 2003; revised 24 February 2004; accepted 12 March 2004

are clearly the most distinctive histological feature of this

tumour.
Malignant EOGCT is exceedingly rare, with only 18
cases reported in the English language literature to date.
The first case appeared in 1971 as a photograph in a WHO
monograph on odontogenic tumours, but no clinical
information was provided in this example.5 The first well
documented case of a malignancy arising in a COC to
appear in the English language literature was reported by
Ikemura et al in 1985.6 Although case reports have
described the malignant tumour using a variety of terms,
including malignant COC,6 8 odontogenic ghost cell
carcinoma,9 11 aggressive epithelial ghost cell odontogenic tumour,4,12 dentinogenic ghost cell ameloblastoma13
and malignant calcifying ghost cell odontogenic tumour,14
all reported cases demonstrated malignant histological
features such as infiltration, cellular pleomorphism,
numerous mitoses and necrosis.
This paper reports four new cases of this malignancy
using the term odontogenic ghost cell carcinoma (OGCC)
and aims at analysing the clinical, radiological and

Odontogenic ghost cell carcinoma

Y Cheng et al

pathological features to compare with those reported in the

English literature (Table 1).
Material and methods
Four cases were retrospectively studied from the Department of Oral and Maxillofacial Surgery, College of
Stomatology, Wuhan University. All cases had been
collected at the hospital during the period 1972 2002
following initial or definitive surgery. Radiographic
examination was performed with panoramic and occlusal
radiography, Waters position and tomography of the
maxilla. Radiographs and clinical follow-up were obtained
from the patient file. The surgical specimens of the initial
presentation and subsequent recurrences were fixed in 4%
buffered formalin and embedded in paraffin prior to routine
haematoxylin and eosin staining.
Two investigators separately evaluated the radiological
features of the four cases. Consultation with another
observer was made if there was any disagreement.
Radiological features were recorded by a majority
decision.
Case reports
Case 1
A 36-year-old Chinese man had been aware of a painless
swelling in his anterior mandible for 2 months. Examination revealed a 5 cm 3 cm 2 cm painless mass in the
right and left mandible. Radiographic examination showed
a well demarcated radiolucency that extended from the first
premolar region of the right mandible to the left lateral
incisor region. All of the involved teeth were mobile and
exhibited irregular root resorption (Figure 1). The teeth
were extracted and a large cyst was enucleated. It was
reported histologically as COC with cystic change. Five
years later, the patient presented with a swelling in the
mandible slightly larger than that at initial presentation.
Examination disclosed a 7 cm 5 cm 2 cm firm, solid,
mandibular mass. Radiographs demonstrated a large,
poorly demarcated mixed radiopaque radiolucent lesion
with destruction of the buccal and lingual cortical plates.
The lesion was treated by mandibular resection.
Histological features of the resected recurrent mandibular mass showed epithelial hyperplasia that had a close
resemblance to the follicular and plexiform pattern of
ameloblastoma and duct-like structures of adenomatoid
odontogenic tumour. Ghost cells and dentinoid in contact
with the odontogenic epithelium were found in the centre
of epithelial masses. Dysplastic epithelium with densely
arranged cells was also found. Most of those cells were
round basaloid cells with nuclear hyperchromatism and
mitosis. Some were polygonal epithelial cells with vascular
nuclei. There were occasional foci of clear cells.
Case 2
A 35-year-old Chinese man sought treatment for a painful
swelling in the right maxilla. Examination revealed a

153

3 cm 3 cm 2 cm painful mass in the right maxilla.

Waters position and occlusal film showed a well
demarcated unicystic radiolucency from the maxillary
right canine to the right second premolar region. The
clinical diagnosis was a periapical cyst and the lesion was
enucleated. At 4 years post operation there was a right
maxillary recurrence. The lesion required more extensive
local resection. A right maxillectomy was performed.
Histologically, the lesion was reported as an ameloblastoma with cystic change. Nine years after the initial
procedure, the patient returned complaining of swelling in
the palatal and nasal region. Clinical and radiological
examination revealed a 10 cm 6 cm 5 cm lesion occupying the right maxillectomy cavity (Figure 2). Computed
tomography (CT) showed a poorly demarcated multilocular, osteolytic radiolucency (Figure 3). A soft tissue
mass occupying the right maxillectomy cavity measured
approximately 13 cm 21 cm. A total maxillectomy was
performed. Histologically, epithelial hyperplasia primarily
formed the follicular pattern of ameloblastoma, but the
plexiform pattern and duct-like structures were also seen.
Ghost cells and dentinoid existed in the centre of epithelial
masses. Epithelial cells with nuclear hyperchromatism and
mitoses were arranged densely in malignant parts of the
lesion. There were occasional foci of clear cells (Figure 4).
Ten years later the patient died of cranial metastasis.
Case 3
A 33-year-old Chinese man complained of tender swelling
on the right side of his face for 6 months. Clinical
examination revealed a 4 cm 2 cm 2 cm painful mass
in the right maxilla. Waters position and occlusal film
showed a well demarcated multilocular radiolucency from
the maxillary right incisor to the right second premolar
region. Root resorption was found (Figure 5). A subtotal
maxillectomy was performed. Histological examination
showed a typical COC in the resection. Five years later the
patient sought treatment for a recurrent lesion in the right
maxilla. Waters position showed a poorly demarcated
osteolytic radiolucency with soft tissue mass occupying the
right maxillectomy cavity. A total maxillectomy was
performed. Histologically, some parts were parenchymal
and some were cystic. The characteristics of pathological
changes in the parenchyma resembled the first case, but the
epithelial arrangement mainly formed duct-like structures,
without clear cells. The malignant characteristics of
epithelial cells in the cystic part were not conspicuous
and several dentinal masses projected towards the fibrous
capsules.
Case 4
A 44-year-old Chinese man had been aware of a painless
swelling in his right mandible for 18 months. Examination
revealed a 3 cm 2 cm 2 cm painless mass in the right
mandible. Lateral oblique projection of the right mandible
showed a well demarcated unilocular radiolucency in the
molar region. Root resorption of the second molar was
visible. The lesion was enucleated. The pathological
diagnosis was calcifying epithelial odontogenic tumour.
He had three recurrences, at 1 year, 4 years and 8 years post
Dentomaxillofacial Radiology

154

Dentomaxillofacial Radiology

Table 1
Case No.

Clinical and radiographic features of reported and new cases of odontogenic ghost cell carcinoma
Authors

Age (years)/sex

Race

Jaw

Pindborg et al

45/M

White Mand.

N/A

N/A

N/A

N/A

N/A

N/A

Ikemura et al

48/F

Asian

Max.

11 18

No

Poorly

Mixed

No

No

3
4
5

Ellis and Shmookler

Ellis and Shmookler
Ellis and Shmookler

55/F
17/M
46/M

Black Mand.
N/A
Max.
White Max.

34 45
11 15
14 16

Yes
No
No

Poorly
Poorly
Poorly

Radiolucent
Mixed
Mixed

No
No
No

No
No
No

6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22

Grodjesk et al
Scott and Wood
McCoy et al
Dubiel-Bigaj et al
Siar & Ng
Alcalde et al
Folpe et al
Lu et al
Lu et al
Lu et al
Lu et al
Kamijo et al
Kim et al
Case 1
Case 2
Case 3
Case 4

46/M
33/M
13/F
42/M
39/M
72/F
20/M
24/M
31/F
19/M
39/M
38/M
33/M
36/M
35/M
33/M
44/M

White
Black
Black
N/A
Asian
Asian
N/A
Asian
Asian
Asian
Asian
Asian
N/A
Asian
Asian
Asian
Asian

11 18
11 18
11 17
14 16
N/A
11 16
11 18
11 18
N/A
37 43
46 48
13 18
46 48
31 46
13 15
11 15
46 48

No
No
No
No
N/A
No
No
No
N/A
Yes
No
No
No
Yes
No
No
No

Poorly
Poorly
Poorly
N/A
N/A
Well
Poorly
Poorly
Poorly
Poorly
Poorly
Well
Poorly
Poorly
Poorly
Poorly
Poorly

Mixed
Mixed
Mixed
N/A
N/A
Mixed
Radiolucent
Mixed
Radiolucent
Mixed
Radiolucent
Mixed
Mixed
Mixed
Mixed
Mixed
Radiolucent

Yes
No
Yes
N/A
N/A
No
No
No
No
No
No
No
Yes
Yes
Yes
Yes
Yes

No
No
Yes
N/A
N/A
No
No
No
Yes
No
No
No
Yes
Yes
No
No
Yes

Max.
Max.
Max.
Max.
Max.
Max.
Max.
Max.
Max.
Mand.
Mand.
Max.
Mand.
Mand.
Max.
Max.
Mand.

M, male; F, female; N/A, not available; Max., maxilla; Mand., mandible; Poorly, poorly defined; Well, well defined

Follow-up
Recurrence and death from
neck extension
Death from intracranial
extension
Recurrence
Recurrence
Same patient reported by
Grodjesk et al
Death from distant metastasis
Recurrence
No recurrence for 7 years
N/A
Recurrence, lost to follow-up
No recurrence
Recurrence
Recurrence, lost to follow-up
No recurrence
Death from local extension
Recurrence
No recurrence for 1 year
No recurrence for 2.5 years
Recurrence
Death from distant metastasis
Recurrence
Recurrence

Odontogenic ghost cell carcinoma

Y Cheng et al

Location Cross midline Border definition Radiolucency Root resorption Tooth displacement

Odontogenic ghost cell carcinoma

Y Cheng et al

155

Figure 1 Case 1. A well demarcated radiolucency that extended from

first premolar region of the right mandible to the left lateral incisor region

operatively. These required more extensive local resection.

Thirteen years after the initial procedure, he returned
complaining of a recurring mass for 2 months. Panoramic
radiography demonstrated a large, poorly demarcated
radiolucency of the mandible from the left second
premolar to the right ramus (Figure 6). Surgery demonstrated destruction of the buccal and lingual cortical plates
of the mandible. Histologically, the characteristics of
pathological changes were the same as the second case.
However, no duct-like structures were found.
Discussion
The malignant counterpart of COC is an exceptionally rare
odontogenic tumour, with only 18 cases previously
reported in the English literature.4 7,9 13,15 18 This paper
presents four new cases of this unusual tumour. Because
the radiological and clinical features of the OGCC have not
been described before, we considered a systematic analysis
was essential.
Although histological criteria have been established for
the diagnosis of the benign COC,5,19 features for the

Figure 2

Case 2. Mixed lesion occupying the right maxillectomy cavity

Figure 3 Case 2. CT showing a poorly demarcated multilocular

radiolucency occupying the right maxillectomy cavity

diagnosis of the malignant form have not yet been

determined. The histological diagnostic criteria of OGCC
is an epithelial lining showing a well defined basal layer of
columnar cells, a layer of cells resembling the stellate
reticulum of the enamel organ, and masses of ghost cells
that may be calcified or not, accompanied by atypical
epithelial cell foci presenting mitosis, keratin pearls,
necrosis and other malignant features. The four cases
presented here fulfilled the criteria of OGCC based on the
histological findings of infiltrative growth, atypical cytological features and ghost cell islands.
Gender distribution of all the reported lesions showed a
male predilection of 3.4:1, although this difference is not
statistically significant. This is different from its benign
counterpart, which has been reported as equally common
in both men and women by other authors.8,14,20,21
The age distribution of the patients in the present study
ranged widely from 13 72 years, with a peak in the fourth
(40.9%) and fifth (27.3%) decades. This is different from
benign COC, which has a peak in the second decade.8,20
This shows that the OGCC occurs approximately two
decades later than the benign COC. The reason may be that
the OGCC apparently arises from malignant transformation of a pre-existing benign COC. The mean age in the
present study was 36.7 years. It is similar to benign COC
(34 years).8,14,20,21 Judging from the number of previously
published cases, it is interesting that the OGCC appears to
be more common in Asians than other races, although the
true prevalence remains unknown. Of the 18 previously
reported cases in the English language literature, where
racial data are provided (14 cases), eight have occurred in
Asians, three in Caucasians and three in Blacks. All four
cases presented here occurred in Asians. The predominance of OGCC in Asians is in keeping with the
demographics of the benign COC, which is also more
common in Asians. This suggests that ethnic origin may
play a role in the incidence of OGCC, but the number of
cases published in the English literature is too small to
draw definite conclusions.
Dentomaxillofacial Radiology

Odontogenic ghost cell carcinoma

Y Cheng et al

156

Figure 4 Case 2. Epithelial cells with nuclear hyperchromatism and mitoses were arranged densely in the malignant parts of the lesion (haematoxylin
and eosin 40 )

Figure 6 Case 4. A large, poorly demarcated radiolucency of mandible

from left second premolar to right ramus

Figure 5 Case 3. A well demarcated multilocular radiolucency from the

maxillary right incisor to the right second premolar region

Of the 22 OGCC reported, 15 (68.2%) were in the

maxilla and 7 (31.8%) in the mandible. There was a clear
predilection of OGCC for the maxilla (ratio 2.1:1),
differing from the site distribution of the benign COC
which occurs equally in both the maxilla and mandible.8,20
In the maxilla, OGCC was not restricted to only one region
such as incisor canine, premolar and molar region, but
rather involved an extensive region. Of the 13 cases in the
maxilla where anatomical location is provided, eight were
located in the incisor canine, premolar and molar region;
three in incisor canine, premolar region; and two in
premolar molar region. Twelve cases showed destruction
of the maxillary sinus with obliteration. The lesions in the
maxilla did not cross the midline. On the other hand, in the
mandible, of the six cases where anatomic location was
Dentomaxillofacial Radiology

provided, three were restricted to the molar region and

three crossed the midline but were located between
bilateral premolar region of mandible.
The OGCC usually appeared as a painful, hard swelling
in the maxilla or mandible with osseous destruction, and
with paresthesia being a frequent finding. It may have
cause expansion of the mandible or maxilla.
Of 22 OGCC, only 19 cases reported radiological
features. From them, most of the lesions (14 cases) appeared
as mixed radiolucent radiopaque lesions. Only a few (five
cases) were totally radiolucent. The border of the OGCC
was usually poorly demarcated except for two cases where it
was a moderately well defined border. Varying degrees of
maxillary sinus involvement, from encroachment to
complete obliteration, have also been described.
It was difficult to make a diagnosis of OGCC based on
radiographic features alone. OGCC showed radiographic
features of a malignant tumour and was not specific. Other
possible diagnoses of malignant tumours included osteosarcoma and malignant ameloblastoma. A diagnosis of
OGCC was possible only after the resected specimen was
examined histologically.
Resorption of the roots of the teeth associated with the
lesion was reported in six cases. Tooth displacement was
seen in four cases. Two cases were associated with an

Odontogenic ghost cell carcinoma

Y Cheng et al

unerupted canine in the maxilla. However, the actual

number of these complications is probably higher, because
the number of OGCC is small and it is possible that some
authors did not describe these features even though they
were seen in radiographs.
At the present time, it is impossible to predict the
biological behaviour of the OGCC. Five of 21 have been
associated with long-term survival without recurrence
following definitive surgery, while 16 of 21 have had a
poor clinical outcome with locally recurrent disease or

157

metastasis (Table 1). Recurrence is common after initial

operation. Therefore, OGCC showed both clinical and
radiographic features of a malignant tumour with high
recurrence.
In summary, this study has systematically analysed the
existing English literature on OGCC and provided precise
information on the clinical and radiographic features of
OGCC. Most of the OGCC appeared as mixed
radiolucent and radiopaque lesions with poorly demarcated
borders.

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