Vol. 127 No.

2 February 2019

Odontogenic tumors and lesions treated in a single

specialist oral and maxillofacial pathology unit in the
United Kingdom in 1992 2016
D1XB.S.M.S. X Siriwardena, BDS, D2X X D3X X
MPhil, PhD, MD (Oral Pathology),a,b Hannah Crane, D4X XBSc, BDS,a
D5XNiall X O’Neill, D6X XBDS, Rasha
a
D7X X D8X X
Abdelkarim, BDS, MMedSci, a

D9XDaniel X J. Brierley, D10X XBDS (Hons), MFDS RCS (Ed), MDFS RCS (Eng), FHEA, FRCPath,a
D1XChristopher X D. Franklin, D12X XBDS, PhD, FDSRCS (Eng), FDSRCS (Edin), FFGDP RCS (Eng), FRCPath,a
D13XPaula X M Farthing, D14X XBSc, BDS, FDSRCS, PhD, FRCPath, FHEA,a
D15XPaul X M. Speight, D16X XBDS, PhD, FDSRCPS, FDSRCS (Eng), FDSRCS (Edin), FRCPath,a and
D17X X
Keith D Hunter, D18X XBSc, BDS, FDS RCSEd, PhD, FRCPath, FHEAa,c

Objectives. Odontogenic tumors (ODTs) are a heterogeneous group of lesions derived from elements of tooth-forming tissues. No
detailed data on the incidence of odontogenic tumors in the United Kingdom have been published. The aim of this study was to
retrospectively describe the range and incidence of odontogenic tumors from 1992 to 2016 in a single specialist unit and to com-
pare this population with others.
Study Design. By using the Oral and Maxillofacial Pathology database, Sheffield (UK), we included both local and referred con-
sultation cases. A proportion of diagnoses were reclassified in accordance with the 2017 World Health Organization
classification.
Results. In total, 559 odontogenic tumors were diagnosed. Overall, the most common lesions were ameloblastoma (196 [33.8%]),
odontoma (148 [25.5%]), and odontogenic myxoma (37 [6.3%]), but this varied between local and referral case populations, with
odontomas being most common in the local population (43%). The sites affected and the gender and age of patients were similar
to other Western populations. Malignant ODTs comprised 33 cases (5.7%), of which 9 (27.3%) were ameloblastic carcinoma.
The majority of the malignant ODTs comprised referral cases.
Conclusions. Here, we present the first detailed data on ODTs within a UK population, and the pattern of incidence from the local
population is similar to other Western populations. The exceptional rarity of malignant ODTs emphasizes the need for specialist
centers for their treatment to gain diagnostic experience. (Oral Surg Oral Med Oral Pathol Oral Radiol 2019;127:151 166)

Odontogenic tumors (ODTs) are a group of hetero- maxillofacial pathologies in the pediatric and adult
geneous lesions that derive from tooth-forming tis- populations in the United Kingdom,6,7 but a detailed
sues.1 These tumors are histologically diverse and epidemiologic study of ODTs has not been reported
comprise a range of lesions from hamartomas to benign as yet.
and malignant neoplasms. Predominantly, they occur The classification of ODTs has evolved over many
within the tooth-bearing areas of the jaws but, rarely, years, but since 1971, the World Health Organization
may present in an extraosseous location as a localized (WHO) has published a unified classification with the
gingival swelling. Several surveys of ODTs have been intention of promoting the use of standard terminol-
published from different parts of the world, and there ogy.8 In 2005, the 3rd edition of the WHO classifica-
is some variation in the distribution of ODTs world- tion included a number of significant changes, such as
wide.2 Ameloblastoma is the most prevalent tumor in the exclusion of the odontogenic cyst and the designa-
Africa and some Asian countries, whereas in Europe tion of the odontogenic keratocyst and the calcifying
and America, odontoma is the most common tumor.3-5 odontogenic cyst as neoplasms.9 After much debate,10
An extensive demographic study by Jones et al. pro- the latest 4th edition of the WHO classification revised
vided a breakdown of the number of different oral and the classification and restored these lesions to the cate-
gory of benign cysts.11,12 In the intervening decade,
a
Unit of Oral and Maxillofacial Medicine, Pathology and Surgery,
School of Clinical Dentistry, University of Sheffield, Sheffield, UK.
b
Department of Oral Pathology, Faculty of Dental Sciences, Univer- Statement of Clinical Relevance
sity of Peradeniya, Sri Lanka.
c
Oral Pathology and Biology, University of Pretoria, South Africa. This article presents the first comprehensive data on
Received for publication May 14, 2018; returned for revision Sep 10,
2018; accepted for publication Sep 23, 2018.
odontogenic tumors from a UK population, under-
Ó 2018 Elsevier Inc. All rights reserved. lining the relative rarity of these lesions and the
2212-4403/$-see front matter importance of access to specialist pathology serv-
http://doi.org/10.1016/j.oooo.2018.09.011 ices for diagnosis.

151
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152 Siriwardena et al. February 2019

studies of the incidence of ODTs have, therefore, been cases (n = 230), odontomas predominated, with 98 cases
confounded by uncertainty regarding the designation (42.6%), compared with 68 ameloblastomas (29.6%).
of some lesions as cysts or tumors. The aim of this Ameloblastoma made up the largest category among the
study was to retrospectively determine the incidence of referred cases (128 of 329 [38.9%]). Malignant ODTs
ODTs in a UK population, according to the 2017 WHO were rare, with only 33 cases (5.9% of all ODTs). The
classification, by using the Oral and Maxillofacial results presented below will focus on the relative inci-
Pathology database, Sheffield (UK), over a 24-year dence of tumors in the local population, with additional
period and to compare this with the published epidemi- comments on the referred cases.
ologic data from various geographic areas across the
world. BENIGN EPITHELIAL ODTS
The mean age of occurrence of ameloblastoma in the
MATERIALS AND METHODS local population was 46.8 § 20 years (range 11 86
The diagnostic database of the department of Oral and years), similar to that for the referral cases (49.2 § 19
Maxillofacial Pathology, Charles Clifford Dental Hos- years). Overall, the male-to-female (M/F) ratio was
pital, Sheffield (UK), was searched for all accessioned 1.5:1, with a male preponderance (Figure 1A). The
cases of ODTs over a 24-year period (1992 2016). All majority of ameloblastomas occurred in the mandible
cases from this period were included, and only those (76%) and 20% in maxilla. The site was not specified
with incomplete data were later excluded upon review. in the remaining cases. This is very similar what has
Local and referred cases were reviewed, and some been seen in other large series of ameloblastoma.13
were reclassified histologically in accordance with the Out of the total 149 ameloblastoma cases in the man-
WHO Classification of Head and Neck Tumors dible (both local and referred), 73.2% were in the pos-
(2017)11 by 2 experienced oral and maxillofacial path- terior segments, and less than 20% were in the anterior
ologists. Clinical information, including patient age mandible (Figure 1B and Figure 1C). Similarly, 75%
and gender as well as the location of the tumor, was of maxillary tumors were found in the posterior region,
recorded. Age data are presented as mean § standard and a small number were reported in the maxillary
deviation (SD). To analyze the site distribution, the sinus (2.6%). The molar region was the most common
mandible was divided into anterior, premolar, molar, site affected in both the mandible and the maxilla.
angle, and ramus areas; the maxilla was divided into Examples of cases are presented in Figures 2 and 3.
anterior, premolar, molar, tuberosity, and antrum areas. The second most common tumor was adenomatoid
Site was determined according to the clinical records ODT (AOT; 7.4% of local cases) and the least common
and by reference to the associated radiology, where was squamous ODT, of which only 2 cases were
available. Cases received from the local population reported (both local cases; Figure 4). Although AOT is
and referral cases were included in the study but were primarily a pediatric tumor, with the majority occur-
also analyzed separately to allow for an estimate of ring between 10 and 20 years (mean age 19.5 § 12),
prevalence in the local population. In cases with multi- the age range was 8 to 63 years. In contrast to amelo-
ple biopsies or recurrences, only the index biopsy was blastoma, AOT showed a slight female predominance
considered. Descriptive statistical analysis of the data (M/F 1:1.14) (Figure 5).
was performed by using the frequencies of categorical Calcifying epithelial odontogenic tumor accounted
variables. Continuous variables were expressed as for 3.5% of the local ODTs (6% of referred cases).
mean, median, and SD. Categorical variables were Overall, there was a male predominance (M/F 1.15:1),
expressed as absolute numbers of cases and percentage and the age range was 18 to 88 years (mean 43.3 §
values. 15.9) (Figure 6A). The mandible was the most com-
mon site (61%; Figure 6B), with the majority of cases
RESULTS in the molar premolar region, followed by the anterior
After excluding cases with incomplete data or inaccu- region. The next most common site was the maxillary
rate diagnostic coding, the final sample comprised 559 premolar region, and 30% of maxillary lesions were
cases (Table I). Overall, there were 526 benign (94.1%) associated with the maxillary antrum (Figure 6C).
and 33 malignant (5.9%) lesions, and the most common Odontogenic gingival epithelial hamartomas are
benign tumors were ameloblastoma (including unicystic believed to originate from remnants of the dental lam-
variant; 196 [37.2%]), followed by odontomas (148 ina and are considered to represent a transitional stage
[28.1%]). The center is a tertiary referral center for oral between a tumor-like growth and a true benign neo-
and maxillofacial pathology, and 329 (59.3%) cases plasm. The diagnostic criteria are not clear. All
were referral ODTs, and 230 (40.7%) were from the archived cases with this diagnosis were reviewed, and
local region, which serves a population of approxi- this diagnosis was supported in only 12 cases (2%)
mately 575,000 (Table II). When considering only local over 24 years.
 Volume 127, Number 2
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Table I. Total number of odontogenic tumors received at the Oral and Maxillofacial Pathology diagnostic service between 1992 and 2016
% of total odontogenic Mean age (years) Age range Male-to-
Diagnosis Total No. tumors (ODTs) (§ standard deviation [SD]) (years) female ratio
Benign Epithelial ODTs
Ameloblastoma 196 33.8 48.4 (§ 19.4) 11 86 1.5:1
Squamous odontogenic tumor (SOT) 2 45 6 83 1:1
Calcifying epithelial odontogenic tumor (CEOT) 28 4.8 45.8 (§ 15.9) 18 88 1.15:1
Adenomatoid odontogenic tumor (AOT) 35 6 19.5 (§ 12.3) 8 63 1:1.14
Benign Mixed Epithelial and Mesenchymal ODTs
Ameloblastic fibroma 8 1.4 15.1 (§ 3.04) 12 21 6:1
Odontoma (total) 148 25.5 18 (§ 13.6) 3 71 1.2:1
Odontoma complex 86 14.8 17.25 (§ 12.8) 3 71 1.2:1
Odontoma compound 62 10.7 19.08 (§ 14.6) 4 66 1:1
Dentinogenic ghost cell tumor 9 1.5 46 (§ 26.5) 5 69 3.5:1
Benign Mesenchymal ODTs
Odontogenic fibroma 24 4.1 39 (§ 15.9) 14 72 1.6:1
Odontogenic myxoma 37 6.3 36.4 (§ 14.3) 1 79 1:1.8
Cementoblastoma 16 2.7 34 (§16.48) 16 83 1:1
Malignant ODTs
Odontogenic carcinomas/sarcomas (total) 33 5.7 54.96 (§ 17.1)
Ameloblastic carcinoma 9 1.5 61.7 (§ 15.9) 26 73 1:1.2
Primary intraosseous squamous cell carcinoma 9 69.5 (§ 28.9) 6 90 1.3:1
Sclerosing odontogenic carcinoma 5 45.2 (§ 8.6) 43 54 1:4
Clear cell odontogenic carcinoma 7 1.2 55 (§ 13.7) 36 76 2.5:1
Ghost cell odontogenic carcinoma 1 57 male
Ameloblastic (odontogenic) carcinosarcoma 2 70.5 64 77 1:1
Other Odontogenic Lesions/ODTs That Are Not Listed in the
4th Edition of WHO8
Odontogenic gingival epithelial hamartoma 12 2 25.8 1 61 3:1

Siriwardena et al. 153

Ameloblastic fibro-odontoma/dentinoma 11 1.9 10 (§ 2.06) 7 14 2.7:1

ORIGINAL ARTICLE
TOTAL 559
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154 Siriwardena et al. February 2019

Table II. Number of ODTs received from local region sources and external/tertiary referrals between 1992 and 2016
Local Referral
Diagnosis Total M/F (No.) Mean age (years) Total M/F (No.) Mean age (years)
Benign Epithelial ODTs
Ameloblastoma 68 47/21 46.8 128 71/57 49.2
Squamous odontogenic tumor (SOT) 2 1/1 45.0
Calcifying epithelial odontogenic tumor (CEOT) 8 4/4 40.8 20 11/9 44.4
Adenomatoid odontogenic tumor (AOT) 17 8/9 18.0 18 6/7 21.4
Benign Mixed Epithelial and Mesenchymal ODTs
Ameloblastic fibroma 8 6/1 15.1
Odontoma (total) 98 50
Odontoma complex 54 30/24 17.5 32 19/13 16.8
Odontoma compound 44 20/24 20.6 18 11/7 15.3
Dentinogenic ghost cell tumor 9 7/2 46
Benign Mesenchymal ODTs
Odontogenic fibroma 11 7/4 34.7 13 8/5 43.4
Odontogenic myxoma 11 4/7 36.5 26 9/17 36.4
Cementoblastoma 4 1/3 49.5 12 8/4 28.6
Malignant ODTs
Ameloblastic carcinoma 1 1/0 72 8 3/5 60.0
Primary intraosseous squamous cell carcinoma 9 2/0 69.5
Sclerosing odontogenic carcinoma 1 0/1 54 4 1/3 43.0
Clear cell odontogenic carcinoma 7 5/2 55.0
Ghost cell odontogenic carcinoma 1 1/0 57
Ameloblastic (odontogenic) carcinosarcoma 2 1/1 70.5
Other Odontogenic Lesions/ODTs That Are Not
Listed in 4th Edition of WHO8
Odontogenic gingival epithelial hamartoma 5 5/0 34.2 7 4/3 19.9
Ameloblastic fibro-odontoma/ dentinoma 4 4/0 10 7 5/2 10.3
TOTAL 230 329

F, female; m, male; ODTs, odontogenic tumors; WHO, World Health Organization.

Fig. 1. A, Age and gender distribution of patients with ameloblastoma. B, Site of occurrence of ameloblastoma. C, Site distribution
of ameloblastoma by percentage, including those confined to a single site and those that extend over a region. Laterality is not
implied by position on the schematic diagram. The body of the mandible and maxilla have been separated into anterior/incisor, pre-
molar, and molar regions, with angle and ramus added for the mandible and tuberosity added for the maxilla. In addition, 1 case was
identified in the hard palate, 9 peripheral tumors were identified (4.6%), and the site was not specified in 8 cases (4.1%).
OOOO ORIGINAL ARTICLE
Volume 127, Number 2 Siriwardena et al. 155

Fig. 2. A representative section of a resection specimen from a white female, age 42 years, with a large multilocular radiolucency in
left body of mandible. Much of the specimen shows conventional ameloblastoma, but there is a central focus with marked
crowding and cytologic atypia. This case was signed out as ameloblastoma with a focus of in situ ameloblastic carcinoma. A high-
resolution version of the image is available as eSlide: VM05372.

Benign mixed epithelial and mesenchymal ODTs from the local population (42.6%) but only comprised
Odontomas, which are hamartomas rather than true 28% of the full cohort. They accounted for 15%
neoplasms, were the most common lesion in the cases of referred cases. Overall, the mean age was

Fig. 3. A representative section of an excisional biopsy specimen from a white male, age 76 years. There was an expansile lesion
of left maxillary tuberosity with no intrabony lesion. A diagnosis of peripheral ameloblastoma was made. A high-resolution ver-
sion of the image is available as eSlide: VM05373.
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156 Siriwardena et al. February 2019

Fig. 4. A representative section of an enucleation specimen from a white female, age 61 years. The “cystic” lesion was adjacent
to, but separate from the retained root of upper right tooth #4 (UR4 A). A diagnosis of a squamous odontogenic tumor was made.
A high-resolution version of the image is available as eSlide: VM05374.

18 § 13.6 years, with a male predominance (M/F evaluated with additional clinical and radiographic
1.7:1). The age range was 3 to 71 years. There was no information (provided by P.M.S. and K.D.H.) to
significant difference between complex and compound exclude developing odontoma and confirm the diagno-
types with respect to age and gender distribution except sis. An example case is shown in Figure 8.
for site, where the complex type mostly occurred in the
posterior mandible (63%) and the compound type in the Benign mesenchymal ODTs
anterior maxilla (81.8%). There was, however, a large In the local population, the most common types were
variation in the site of occurrence, and odontomas were odontogenic myxoma and odontogenic fibroma (both
diagnosed in every segment of both jaws. A number of 4.8%). Odontogenic myxoma occurred in a wide age
complex odontomas did occur in the anterior maxilla range (1 79 years), with most patients age between 31
(16%), perhaps indicative of the variation in histologic and 40 years (mean age 36.4 § 14.3 years; Figure 9A).
features and the difficulties in precise classification of Of the odontogenic myxomas, 68.8% were found in
odontomas if they do not present as discrete denticles. the mandible (Figure 9B), and most occurred in the
A total of 30 lesions that contained ghost cells as a premolar and molar areas (40.6%). However, not all
significant/prominent histologic feature were identified lesions were confined to a single subsite because most
in the database, and all were reviewed. Of these, only 9 tumors were large at presentation (Figure 9C). A total
met the diagnostic criteria for dentinogenic ghost cell of 16 cementoblastomas were diagnosed, all of which
tumor (see Table I; Figure 7), all of which were were in the mandible, with 13 (81%) attached to the
referred cases (see Table II). Because calcifying odon- first or second permanent molar.
togenic cysts have been reinstated as odontogenic cysts
in the 4th edition of the WHO classification, these Malignant ODTs
were not included in our data. We did not identify any Malignant ODTs were rare, with just 33 cases overall
primordial ODTs on our review of the cases. (5.7% of all ODTs). Only 2 of these cases came from
Although ameloblastic fibro-odontoma/dentinoma the local population (<1% of total population)—1
have been removed from the 2017 ODT classification, ameloblastic carcinoma, and 1 sclerosing odontogenic
we identified 11 cases with a male predominance (M: F carcinoma. Overall, there were 9 cases each of amelo-
4:1). Age range was 7 to 14 years, with a mean age of blastic carcinoma and primary intraosseous carcinoma
10 § 2.1 years. The majority (7 of 11) of these cases (see Tables I and II). Seven of the 9 intraosseous carci-
were referrals. These 2 subtypes of ODTs were re- nomas occurred in association with an odontogenic
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Volume 127, Number 2 Siriwardena et al. 157

Fig. 5. A, Age and gender distribution of patients with adenomatoid odontogenic tumour. B, Site of occurrence of adenomatoid
odontogenic tumor. C, Site distribution of adenomatoid odontogenic tumor by percentage. The body of the mandible and the max-
illa have been separated into anterior/incisor, premolar, and molar regions, with angle and ramus added for the mandible and
tuberosity added for the maxilla. The site was not specified for 2 cases (5.7%).

cyst. The mean age of all the patients with malignant demographic study of all odontogenic pathology per-
tumors was 54.96 § 17.1 years (Figure 10A). The man- formed in our department reviewed specimens
dible was most commonly affected (Figure 10B), and received over a 30-year period. Out of 44,007 speci-
the majority of cases involved the whole body of the mens, only 331 were diagnosed as ODTs (0.75%).6,7
mandible (28%; Figure 10C). Example cases are shown A similar proportion was also seen in the United
in Figures 11 and 12. States, where ODTs accounted for 1.2% of all cases
over a 20-year period.5 The benefit of the present
Referred cases study is that it included data from both local hospitals
The relative proportions of lesions that were referred to and referrals from other centers, with the referrals
the unit were different from the local population (see contributing to a greater proportion of the ODT diag-
Table II). The proportion of odontomas was much noses (59.3%). It could cautiously be argued that once
lower in the referred population, and consequently the the referred cases are removed, the local data are
proportion of ameloblastomas was much higher (see reflective of the relative incidence of these lesions
Table II). There was also bias toward the rarer tumors across the United Kingdom; indeed, the pattern of
and those in which diagnostic difficulties were well incidence is very similar to that described in other
described, including the malignant tumors, 94% of Western populations.
which were referrals. The referral data were removed for much of the
analysis because including it would have introduced
DISCUSSION bias toward that population. This separation of the
This study is the first to look in detail at the relative cohort into local cases served by the diagnostic ser-
frequency of ODTs in a UK population. A previous vice and those accessioned as referrals is informative
ORAL AND MAXILLOFACIAL PATHOLOGY OOOO
158 Siriwardena et al. February 2019

Fig. 6. A, Age and gender distribution of calcifying epithelial odontogenic tumor (CEOT). B, Site of occurrence of CEOT. C, Site
distribution of CEOT by percentage, including those confined to a single site and those that extend over a region. The body of the
mandible and the maxilla have been separated into anterior/incisor, premolar, and molar regions, with angle and ramus added for
the mandible. The site was not specified for 2 cases (7%).

Fig. 7. A representative section of an excision biopsy for a clinical diagnosis of a fibrous epulis in a 49-year-old, white male. The
lesions was a gingival swelling between lower right tooth #4 (LR4) and LR5. The features are those of a peripheral dentinogenic
ghost cell tumor. There was no intrabony component. A high-resolution version of the image is available as eSlide: VM05375.
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Volume 127, Number 2 Siriwardena et al. 159

Fig. 8. A radiologic image (A) and representative section of an enucleation specimen (B) from a lesion in the mandible in a white
male, age 12 years. This was an extensive multilocular lesion of the right ramus, with gross expansion and loss of cortication of
the mandible in areas. The lesion enucleated intact. A diagnosis of ameloblastic fibro-odontoma was made in this case, and this
diagnosis was upheld in the review process outlined in this article. A high-resolution version of the image is available as eSlide:
VM05376.
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160 Siriwardena et al. February 2019

Fig. 9. A, Age and gender distribution of patients with odontogenic myxoma (n = 37). B, Site of occurrence of odontogenic myx-
oma. C, Site distribution of odontogenic myxoma by percentage, including those confined to a single site and those that extend
over a region. The body of the mandible and the maxilla have been separated into anterior/incisor, premolar, and molar regions
with angle and ramus added for the mandible. The exact site was not specified in 5 cases (13.5%).

for a number of reasons. The distribution of cases in variability are complex, and although there may be
the referred population is very different (see Table II). true differences in the incidence of ODTs in differ-
This reflects the fact that the referred cases were ent populations, some element of the variation may
biased toward the rarer and diagnostically more diffi- have resulted from differences in the submission of
cult tumors, and they reflected areas of difficulty in lesion samples for histologic examination. In some
diagnosis that often prompt an onward specialty circumstances, a diagnosis of odontoma may be
referral.14 made on the basis of radiologic appearance only,
The most common tumor overall was ameloblas- and therefore, the tissue may not be sent for histo-
toma, comprising 33.8% of the total number of logic examination: Indeed, many of the case series
ODTs, but this is not the case when only consider- come from populations in the developing world,
ing the local cases, where odontomas predominated where access to histopathology services may be
(43%). The relative incidence of these lesions is limited.
also largely determined by the age of the patient, as Despite the removal of ameloblastic fibro-odontoma
demonstrated by Jones and Franklin, who reported (AFO) and ameloblastic fibrodentinoma from the
ameloblastoma to be the most common tumor 2017 WHO classification, we have reported these
among adults, comprising 30.8%,7 but less than 5% separately in Tables I and II. The nature of the
in children, among whom odontomas predominated mixed ODTs, including ameloblastic fibroma (AF),
(76%).6 Ameloblastoma has been reported as the has been a subject of debate and disagreement for
most frequent ODT in Turkey, 2 Egypt, 15and glob- some years. 17 It is widely accepted that AF is an
ally, as reviewed in a study that reported a world- entity in its own right because the mean age is
wide frequency of 8544 ODTs. 16 However, other higher than that for AFO, which does not support
studies, such as that carried out in the United States the concept of an AF AFO odontoma continuum.
(California), identified odontomas as the most fre- The issue with the relationship between AFO and
quently occurring ODTs. 5 The reasons for this odontoma is more problematic. AFOs are often
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Volume 127, Number 2 Siriwardena et al. 161

Fig. 10. A, Age and gender distribution of patient with malignant odontogenic tumors (ODTs). B, Site of occurrence of malignant
ODTs. C, Site distribution of malignant ODTs (n = 33) by percentage, including those confined to a single site and those that
extend over a region. The body of the mandible and the maxilla have been separated into anterior/incisor, premolar, and molar
regions, with angle and ramus added for the mandible. One case primarily involved the maxillary antrum. The site was not speci-
fied in 6 cases (18%), and 1 lesion was solely within maxillary gingiva (3%).

Fig. 11. A representative section from an incisional biopsy from a 72-year-old male. There was a large and destructive lesion in
the left anterior/body of mandible. The diagnosis of ameloblastic carcinoma was made. A high-resolution version of the image is
available as eSlide: VM05377.
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162 Siriwardena et al. February 2019

Fig. 12. A representative section from an excision specimen from a female, age 76 years. There was an extensive, destructive
lesion of left anterior maxilla. Cytogenetic analysis revealed EWSR1 gene rearrangements. The final diagnosis was of a clear cell
odontogenic carcinoma. A high-resolution version of the image is available as eSlide: VM05378.

histologically indistinguishable from developing original introduction in 2005 caused an increase in

odontomas, and the vast majority of them are best the overall rates of frequency and prevalence of
regarded as developing odontomas. However, large, ODTs.23 Studies in populations that are directly
destructive AFOs have been reported, 18,19 and this comparable with our cohort are very few, but in gen-
may indicate that there is a spectrum of biologic eral, the patterns of ODT incidence are very similar
potential. Although most are probably hamartomas, to that seen in our local population. In Western popu-
some may be truly neoplastic. lations, odontomas are the most common ODT, fol-
Table III4-37 shows a summary of published case lowed by ameloblastomas, although there is a
series from around the world. Direct comparisons marked variability in the relative proportions.5,24
between the present study and previous studies are
difficult to make. Multiple reasons for this exist, and
although this is the first study that has looked at the CONCLUSIONS
frequency of ODTs in line with the 2017 WHO clas- Malignant tumors comprise a very small proportion
sification,11 we have attempted to make the data as of ODTs. Only 6% of all ODTs were malignant,
comparable as possible—for example, by removing most of them accessioned as referral cases (31 of
the keratocystic odontogenic tumor and the calcify- 33). The fact that these lesions are quite rare empha-
ing cystic odontogenic tumor from the data. We sizes the need for access to specialist expertise to
identified 3 similar studies published in 2017; how- gain experience in diagnosing such lesions. This has
ever, these studies assessed the prevalence of ODTs implications for the provision of training of oral and
in line with the 2005 WHO classification.20-22 The maxillofacial pathologists and in the maintenance of
2017 WHO classification made some substantial expertise, given that the number of malignant ODTs
changes to the classification of ODTs, including diagnosed has been significantly low. It is, thus,
reclassification of the keratocystic odontogenic important that the centers that have experience in the
tumor as an odontogenic keratocyst and removing it management of these tumors (local or referred cases)
from the tumor category.11 During the period of this make data on these cases available for training and
study, 374 odontogenic keratocysts were diagnosed continuing education so that the expertise in the
in Sheffield (UK). This would have had a marked diagnoses of these exceedingly rare tumors can be
effect on the relative frequency of ODTs because its accumulated.
 Volume 127, Number 2
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Table III. A summary of studies that have reported incidence of odontogenic tumors (ODTs)

UK (children)6
Saudi Arabia25

Rural Africa20
Present study

*Nigeria42-44

UK (adult)7
Argentina49
*Libya40, 41

*Brazil45-47
*Turkey2, 28

Sri Lanka4

Thailand38
*China3, 37
Pakistan36
*India29-35

Ethiopia22

Estonia24
Mexico23
*Iran26, 27

Africa39

Egypt15

Chile48

USA5
Benign Epithelial
ODTs
Ameloblastoma 30% 43% 53% 32% 62% 79% 68% 63% 59% 54% 59% 80% 52% 46% 70% 37% 25% 21% 20% 12% 25% 41% 5%
Squamous Odon- <1% 1% 1% 1%
togenic Tumor
(SOT)
Calcifying epithe- 3% 2% 1% 11% 2% 2% 1% 1% 2% 1% 5% 2% 1% 1% 1% 1% 1% 1% 5%
lial odontogenic
tumor (CEOT)
Adenomatoid 7% 7% 6% 2% 8% 6% 6% 5% 5% 6% 4% 5% 3% 4% 6% 6% 7% 5% 2% 1% 2% 6%
odontogenic
tumor (AOT)
Benign Mixed
Epithelial and
Mesenchymal
Tumors
Ameloblastic 1% 2% 1% 2% 1% 1% 1% 6% 2% 3% 1% 3% 2% 1% 2% 2% 16% 5%
fibroma
Odontoma (total) 43% 26% 21% 31% 19% 6% 14% 10% 22% 4% 7% 3% 16% 28% 2% 39% 48% 48% 53% 78% 35% 32% 76%
(Ameloblastic 2% 1% 3% 2% 1% 2%
fibrodentinoma)
(Ameloblastic 2% 3% 2% 1% 1% 1% 1% 2% 5% 3%
fibro-odontoma)
Dentinogenic 1% 1%
ghost cell tumor
Benign Mesen-
chymal
Tumors
Odontogenic 5% 1% 1% 3% 2% 2% 1% 2% 1% 7% 5% 2% 1% 3% 2% 5% 6% 3% 1% 2% 2%
fibroma

Siriwardena et al. 163

Odontogenic 5% 11% 10% 6% 2% 4% 5% 6% 5% 10% 10% 6% 10% 8% 12% 9% 14% 10% 8% 2% 12% 1% 3%

ORIGINAL ARTICLE
myxoma
Cementoblastoma 2% 3% 2% 2% 1% 3% 2% 3% 5% 2% 3% 2% 2% 1% 8% 1% 1%
Malignant ODTs
Odontogenic car- <1% 2% 2% 8% 2% 2% 7% 1% 19% 4% 1% 3% 1% 3% 1% 1%
cinomas/sarco-
mas (total)
(continued on next page)
 164 Siriwardena et al.
ORAL AND MAXILLOFACIAL PATHOLOGY
Table III. Continued

UK (children)6
Saudi Arabia25

Rural Africa20
Present study

*Nigeria42-44

UK (adult)7
Argentina49
*Libya40, 41

*Brazil45-47
*Turkey2, 28

Sri Lanka4

Thailand38
*China3, 37
Pakistan36
*India29-35

Ethiopia22

Estonia24
Mexico23
*Iran26, 27

Africa39

Egypt15

Chile48

USA5
Malignant/metas- 2%
tasizing
ameloblastoma
Other Odonto-
genic Lesions
Odontogenic 2% 5%
hamartoma
Odonto- 1%
ameloblastoma
Total Number of 230 119 469 232 1027 142 1247 1854 445 140 572 3034 66 178 892 426 410 362 153 1087 75 262 175
Cases
Note: The data for the present series is the local data only, to allow for comparisons.
*In some cases, the individual cohorts have been combined if they come from the same country, and the mean values are stated. Other than for the current cohort, all of the assessment of the tumors was com-
pleted by using the WHO Classification, 3rd edition (2005). KCOT (odontogenic keratocyst) and CCOT (calcifying odontogenic cyst) have been removed from the published totals, where appropriate.

February 2019
OOOO
OOOO ORIGINAL ARTICLE
Volume 127, Number 2 Siriwardena et al. 165

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