Sun et al.

Diagnostic Pathology (2019) 14:92

https://doi.org/10.1186/s13000-019-0867-4

CASE REPORT Open Access

Primordial odontogenic tumor: a case

report and literature review
Qiaochu Sun1, Jae-Seo Lee2, Okjoon Kim1 and Young Kim1*

Abstract
Background: A primordial odontogenic tumor (POT) is a rare, benign, mixed epithelial and mesenchymal
odontogenic tumor that has been included as a new entity in the latest World Health Organization (WHO)
classification (2017). POT consists of dental papilla-like myxoid connective tissue covered with a delicate membrane
of ameloblastic epithelium. Only 15 cases have been documented worldwide, and here, we report the sixteenth
case and the first one of South Korea.
Case presentation: An asymptomatic lesion was discovered as an incidental radiographic finding in a 10-year-old
boy. The patient had no complaints about the lesion. Cone-beam computerized tomograms revealed a round
cavity with a defined cortical border measuring approximately 5 × 5 × 5 mm in size. The lesion was a POT. The
patient was treated with enucleation. The tumor showed no recurrence for one year.
Conclusion: This is the first report of POT in South Korea using the novel diagnosis of POT after it was recognized
and defined in the latest WHO classification. This novel diagnosis will be useful for pathologists and clinicians in
diagnosing and differentiating this new and rare disease from other odontogenic tumors.
Keywords: Primordial odontogenic tumor, Odontogenic tumors, Odontogenesis

Background in a cuboidal-to-columnar epithelium and resembling

A primordial odontogenic tumor (POT) is a new entity the inner epithelium of the enamel organ [2]. Bologna-
classified as a benign, mixed odontogenic tumor in the Molina et al. investigated the possible histogenesis and
fourth edition of the World Health Organization (WHO) biological behavior of POTs using various immunohisto-
classification of Head and Neck Tumors in 2017 [1]. Mos- chemical methods and suggested that POT is a benign,
queda-Taylor et al. (2014) analyzed the clinicopathological odontogenic tumor that develops during the primordial
and immunohistochemical features in a series of six cases stage of tooth development [4].
that did not fulfill the previous criteria for odontogenic tu- Until now, only 15 cases have been documented
mors [2], and the term “primordial odontogenic tumor” worldwide [5–7]. To better understand this novel entity
was first used to describe the novel lesion. to diagnose it correctly, we report the sixteenth case
To date, most cases of POT were found as well-de- worldwide and the first case of POT in Korea since it
fined unilocular or multilocular radiolucent lesions adja- was defined in the latest WHO classification.
cent to the crown of an unerupted tooth. Patients
showed asymptomatic bone swelling, producing root re-
sorption, and buccal or lingual cortical expansion. Case presentation
Macroscopically, the tumor is a pale, slippery, solid nod- A 10-year-old healthy boy visited the Department of
ule that tends to be encapsulated [2, 3]. Histopathologic- Pediatric Dentistry, Chonnam National University in July
ally, POTs consist of variably cellular-to-loose fibrous 2018 to complete root canal therapy. An asymptomatic
tissue with dental papilla-like areas, entirely enveloped lesion was discovered incidentally in a conventional
panoramic X-ray. There was no history of trauma to the
* Correspondence: [email protected] area and he had no complaints about the lesion. There
1
Department of Oral Pathology, School of Dentistry, Chonnam National were no abnormal findings in either the physical exam-
University, 77 Yongbong-ro, Buk-gu, Gwangju 61186, Republic of Korea
Full list of author information is available at the end of the article
ination or laboratory data.

© The Author(s). 2019 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0
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reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to
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Sun et al. Diagnostic Pathology (2019) 14:92 Page 2 of 8

Cone-beam computerized tomograms depicted a of the tumor was composed of loose and myxoid fibrous
round cavity with a defined cortical border measuring tissue, including spindle cells (Fig. 2a, b). In some areas, the
approximately 5 × 5 × 5 mm in size, mesiolingual to the cords or islands of the epithelium were observed in the
root of tooth 34 (Fig. 1a). A panoramic radiograph connective tissue because of tangential folded sectioning.
showed a periapical bone resorption with sclerosing os- The cord-like or nests of the enfolded epithelium possessed
teitis on the apical to the adjacent tooth (Fig. 1b). stellate reticulum between the columnar cells (Fig. 2c).
The diagnostic hypotheses from radiology were simple Dentine was found in the peripheral portion of the con-
bone cyst, periapical cemental dysplasia, and paradental nective tissue (Fig. 2d), which represents an association be-
cyst because of its location and radiologic features. A tween tumor and adjacent tooth.
tumor enucleation was performed, and a whitish, firm, Upon immunohistochemistry analysis, the epithelial
myxoid connective tissue was transferred for pathological component demonstrated strong positivity for cytokera-
analysis. Based on the histopathological study results, tin 19 only in columnar cells, which was consistent with
POT was confirmed as a definitive diagnosis. There were previously reported cases. Vimentin was also positive
no adverse events neither signs of recurrence after surgery throughout the tumor tissue; specifically, strongly posi-
during a one-year follow-up. tive in the epithelial layers and moderately positive in
The gross specimen showed a 5 × 5 × 5-mm-sized well- mesenchymal tumor cells. In addition, alpha smooth
defined and slippery white nodule of pale, translucent, firm, muscle actin (α-SMA) and S100 protein were negative in
myxoid connective tissue. Histologically, the periphery of the ectomesenchymal cells. Ki67 expression was lower
the tumor was enveloped by a delicate membrane of amelo- than 2% (Fig. 3).
blastic epithelium, which is a single layer of columnar epi-
thelium exhibiting typical “reverse nuclear polarization,” Discussion and conclusions
i.e., displacement of nuclei away from the basement mem- POT is a new neoplastic entity, classified as a benign,
brane and vacuolated cytoplasm at the bottom part. Most mixed epithelial and mesenchymal odontogenic tumor

Fig. 1 Radiographic findings of POT. a Cone-beam CT showing a round cavity with defined cortical border that is mesiolingual to the root of
tooth 34. b-d Panoramic radiograph demonstrates well-defined radiolucency (circled area) in the mandibular left region
Sun et al. Diagnostic Pathology (2019) 14:92 Page 3 of 8

Fig. 2 Microscopic findings of the POT. a It consisted of a proliferation of cellular myxoid connective tissue, which is less vascular, less cellular,
and more collagenous. The periphery of the tumor is surfaced by a monolayer of columnar epithelium. The cord-like or nests of enfolded
epithelium are present (hematoxylin and eosin stain; magnification, × 40). b The external aspect of the tumor is surfaced by columnar epithelial
cells, which show “reverse nuclear polarization” (nuclei displaced away from the connective tissue and cytoplasm showed vacuolated at the
bottom part) (magnification, × 200). c The cord-like or nests of the enfolded epithelium possessed stellate reticulum between the columnar cells
(magnification, × 200). d Dentine existed adjacent to the tumor (magnification, × 40)

Fig. 3 Histopathological and immunohistochemical findings of POT. a Hematoxylin and eosin staining of POT. b CK19 was positive only in
columnar epithelium. c Vimentin was positive throughout the tumor tissue (strongly positive in epithelial layers and moderately positive in
mesenchymal tumor cells). d Ectomesenchymal cells were negative for α-SMA. e Ectomesenchymal cells were negative for S-100 protein. f Ki67
labeling index was basically lower than 2%. (magnification, × 100)
Sun et al. Diagnostic Pathology (2019) 14:92 Page 4 of 8

in the fourth edition of the head and neck WHO blue stage (8–10 years), and nine cases (56.25%) in the per-
book in 2017 [1]. The term “POT” was first established manent dentition stage (13–19 years). Most cases, in-
by Mosqueda-Taylor et al. in 2014 [2], and the authors cluding this case (14/16, 87.5%), occurred in the
reported a series of six cases clinicopathologically and mandible, and the remaining two cases occurred in the
immunohistochemically. maxilla. The prognosis of all POTs was excellent after
Until now, there had been only 15 cases of POT re- surgery, except for two cases which were lost to follow-
ported in the literature. The previously reported cases up, recurrences of all reported cases have not been re-
share similar clinical and radiological findings, which are ported to date (median follow-up years = 4.53 ± 6.09,
shown in Table 1. Regarding the statistics of all of these ranging from 3 months to 20 years). In our case, until
cases, including the current one, there were ten males now after enucleation, there was no recurrence either. It
(62.5%) and six females (37.5%). The median age was seems that enucleation and extraction of involved tooth
11.3 years for all 16 cases (ranging from 2 to 19 years). were effective treatments because the peripheral colum-
The dentition stages of the patients are as follows: five nar epithelium or fibrous pseudocapsule of the tumor
cases (31.25%) affecting the deciduous dentition stage clearly delimited the boundaries of the tumor from adja-
(2–5 years), two cases (12.5%) during mixed dentition cent tissues. It is worth noting that the geographic

Table 1 Summary of previous and current reports of primordial odontogenic tumor

Case Age (years)/ Location Clinical findings Radiographic Treatments Follow-up References
No. gender findings
1 18/M Mandible Asymptomatic, buccal swelling. RL, UL, well-defined, Enucleation and 20 years, Mosqueda-Taylor
Clinically evident for 6 months 45 × 40 mm tooth extraction NED et al. (2014) [2]
2 16/M Mandible Asymptomatic, buccal and inferior RL, UL, well-defined, Enucleation and Follow-up Mosqueda-Taylor
mandibular cortical bone expansion. 55 × 50 mm tooth extraction lost et al. (2014) [2]
Clinically evident for 4 months
3 16/M Mandible Asymptomatic, buccal swelling. RL, UL, well-defined, Enucleation and 10 years, Mosqueda-Taylor
Clinically evident for 1 year 65 × 50 mm tooth extraction NED et al. (2014) [2]
4 3/F Mandible Asymptomatic, buccal and lingual RL, biloculated, well- Enucleation and 9 years, Mosqueda-Taylor
bony expansion. Clinically evident defined, 90 × 70 mm tooth extraction NED et al. (2014) [2]
for 22 months
5 13/F Mandible Asymptomatic, buccal swelling. RL, biloculated, well- Enucleation and 3 years, Mosqueda-Taylor
Clinically evident for 4 months defined, 80 × 50 mm tooth extraction NED et al. (2014) [2]
6 3/F Maxilla Asymptomatic, buccal and palatal RL, UL, well-defined, Enucleation and 6 months, Mosqueda-Taylor
bony swelling. Clinically evident 35 × 30 mm tooth extraction NED et al. (2014) [2]
for 3 months
7 19/M Mandible Asymptomatic, buccal and lingual RL, UL, well-defined, Excision and tooth 7 months, Slater LJ et al.
swelling 25 × 19 mm extraction NED (2016) [3]
8 8/F Maxilla Asymptomatic, buccal swelling RL, UL, well-defined, Enucleation 16 months, Ando et al.
16 × 15 mm NED (2017) [8]
9 5/M Mandible Asymptomatic, buccal swelling RL, UL, well-defined, Excision and tooth 7 months, Mikami et al.
80 × 80 mm extraction NED (2017) [9]
10 17/M Mandible Asymptomatic, swelling RL, multilocular, well- Enucleation and 6 months, Bajpai and Pardhe
defined, 30 × 20 mm tooth extraction NED (2018) [10]
11 15/F Mandible Slight fullness of the right mandibular RL, multilocular, well- Excision and tooth 3 months, Asma Almazyad
vestibule defined, 35 × 20 mm extraction NED et al. (2018) [11]
12 18/M Mandible Asymptomatic, incidentally RL, UL, well-defined, Curettage and tooth 20 months, Asma Almazyad
noted intra-osseous lesion 12 × 7 mm extraction NED et al. (2018) [11]
13 2/M Mandible Asymptomatic, swelling RL, multilocular, well- Excision and tooth 2 years, Hatem Amer
defined, 30 × 40 mm extraction NED et al. (2018) [5]
14 4/M Mandible Asymptomatic, buccal and lingual RL, UL, well-defined, Enucleation and Follow-up Bomfim B B
bony expansion. Clinically evident 30 × 20 mm tooth extraction lost et al. (2018) [6]
for 8 months
15 13/F Mandible Asymptomatic, volume augmentation. RL, UL, well-defined Enucleation and 13 years, Teixeira L N
Clinically evident for 3 months tooth extraction NED et al. (2019) [7]
16 10/M Mandible Asymptomatic UL, well-defined, Enucleation one year, Present case
5 × 5 mm NED
NED no evidence of disease, RL radiolucent, UL unilocular
Sun et al. Diagnostic Pathology (2019) 14:92 Page 5 of 8

regions of the POT cases were mainly located in North analyzed the size of every case classified by location type
and South America (68.8%). Only 25% of the reported as described above. The size of Type A POT ranged
cases (including the current case) occurred in Asia, and from 12 mm to 90 mm and Type B ranged from 25 mm
there was only one case reported in Egypt, Africa. POTs to 80 mm. There is only one case of Type C POT (the
may occur at a higher incidence in Western countries, present case), and its size was 5 mm. Further evaluation
but greater numbers of cases are needed to further dem- is needed to determine whether a Type C tumor charac-
onstrate this and study the etiology of POTs. teristically shows a smaller size than that of Type A and
The present case showed a rare location with a small Type B. The number of reported cases is not large
tumor, whereas clinical, radiological, and pathologic enough, therefore, greater numbers of POT cases are re-
findings are similar to those of the previous reported quired to obtain a better understanding of this rare
cases. Interestingly, the location of this tumor was near entity.
the root of the tooth, whereas previously reported cases In histological findings, our case was enveloped by a
presented as a pericoronal location in close association single layer of columnar epithelium exhibiting typical
with unerupted teeth. To verify a relationship between “reverse nuclear polarization”, which is known as amelo-
the patient’s dentition stage and the location of the blastic epithelium. In some areas, the cord-like or islands
tumor, we summarized the location of all POTs and of the enfolded epithelium possessed stellate reticulum
listed the information in a schematic diagram. We then between the columnar cells. Calcification was found in
attempted to classify the location of POT by three types the peripheral portion of the myxoid connective tissue,
based on the previous literature (Fig. 4): Type A, the which represents an association between the tumor and
POT has a pericoronal location in a dentigerous rela- adjacent tooth. The pathological characteristics in our
tionship; Type B, the tumor appears to completely en- case are conclusive enough to make a diagnosis [3, 8].
velop an embedded tooth; and Type C, the POT is in According to Mosqueda-Taylor et al. [2], who first de-
close proximity to the root of the tooth. There were 12 scribed and termed this new entity, POT should be care-
Type A cases; four of them (33.3%) were in the stage of fully distinguished from an ameloblastic fibroma (AF),
deciduous dentition, one of them (8.3%) was in the central odontogenic fibroma (COF), and odontogenic
mixed dentition stage, and the remaining seven (58.3%) myxoma (OM). An AF can be easily differentiated from
were in the permanent dentition stage. Three cases fit POT because the histological picture is quite different.
the criteria for Type B; one of them was in the decidu- An AF is a tumor composed of odontogenic ectome-
ous dentition stage, and the other two cases were in the senchyme resembling the dental papilla with epithelial
permanent dentition stage. Only one case was Type C cords and immature mesenchymal stroma without den-
(the present case), and it was found in the mixed denti- tal hard tissues [12]. POT also contains a small number
tion stage (Table 2). In every dentition stage, Type A of cord-like epithelium or nests, which is similar to an
was the most common. The current case is a unique re- AF; however, the epithelial cords or nests of POTs are
port of the first Type C case worldwide. It appears that present in a limited area near the periphery of the tumor
the patient’s dentition stage is not determined by the lo- [8]. Moreover, the mesenchymal components in AF
cation type of the POT, although it will be necessary to cases are more cellular, and the ameloblastomatous epi-
evaluate more cases. In addition, the POT size in the thelial component is conspicuous [2, 13, 14], whereas
present case was the smallest compared with that in pre- ameloblastomatous islands are not seen in the main
viously reported cases (ranging from 12 mm to 90 mm) body of POTs [11]. A COF is an infrequently reported
(Table 1). To determine whether there is a relationship tumor accounting for only 0.1% of all odontogenic tu-
between the size of the POT and the location type, we mors [15–18]. When comparing the pathological

Fig. 4 Schematic overview of the POT location and involved tooth. a Type A, POT has a pericoronal location in a dentigerous relationship. b Type
B, the tumor appears to completely envelop an embedded tooth. c Type C, the POT is in close proximity to the root of the tooth
Sun et al. Diagnostic Pathology (2019) 14:92 Page 6 of 8

Table 2 Relationship of different POT types and dentition stage

Type Deciduous dentition stage Mixed dentition stage Permanent dentition stage Total cases No.
Aa 4/12 (33.3%) 1/12 (8.3%) 7/12 (58.3%) 12
Bb 1/3 (33.3%) 0/3 (0%) 2/3 (66.7%) 3
c
C 0/1 (0%) 1/1 (100%) 0/1 (0%) 1
a
Type a, POT has a pericoronal location in a dentigerous relationship
b
Type B, the tumor appears to completely envelop an embedded tooth
c
Type C, the POT is in close proximity to the root of the tooth

features of both entities, COFs have been categorized origin [28]. In the present case, vimentin was strongly
into two types: an epithelium-poor (simple type) and an positive in epithelial cells and moderately positive in
epithelium-rich type (WHO type) [18], but it does not mesenchymal cells. Kero et al. [29] demonstrated that
show an external covering of ameloblastic epithelium as vimentin was positively expressed in dental epithelium
in POTs. An OM is a rare, benign tumor of odontogenic of the enamel organ between the tenth and twentieth
mesenchymal origin. Radiographically, OMs present as a gestational week, but there was no detectable vimen-
frequently multilocular radiolucent lesion [19]. The tin expression after 27 gestational weeks [30]. These
“soap-bubble, honeycomb, or tennis-racket trabecula- data suggest that the POT may occur at the primor-
tion” radiological image can be found in OMs [20–25], dial stage of tooth development around the tenth-
but the association between OMs and impacted teeth twentieth week (cap-to-bell stage). Besides, the pres-
are rarely found to differ from POTs [2–5, 8, 10, 11, 26]. ence of the transcription factor PITX2 in POT tu-
Pathologically, unlike POTs, an OM is never enveloped moral epithelium, also supports the hypothesis that
by ameloblastic epithelium [3]. this tumor probably derived from the early stages of
Among the 16 cases (including this case), only 11 dental morphogenesis [4]. Teixeira L N et al. [7]
cases have been analyzed immunohistochemically mentioned that the expression pattern of cytokeratin
(Table 3). Specifically, 10 cases underwent immuno- 18 in the inner enamel epithelium-like epithelium of
histochemical analysis for CK19, and in all cases, POT and that of vimentin in the whole tumor might
CK19 was positive in the epithelium of the tumor be important to investigate tumor pathogenesis. This
(predominated in the columnar epithelium). Nine pattern of cytokeratin 18 and vimentin are also ob-
cases were analyzed for vimentin, α-SMA, and S-100 served during tooth development, which reinforces
protein, and in 9/9 (100%) of the cases, vimentin was the theory that POT is derived from a primordial
positive throughout the tumor tissue, while α-SMA tooth germ [7]. Consistent with previously reported
and S-100 protein were not expressed. Eight cases cases, α-SMA and the S-100 protein were negative in mes-
were analyzed for Ki67, which was lower than 2%. enchymal tumor cells [2, 8, 9]. To quantify the prolifera-
CK19 is often positively expressed in the epithelium tive activity of the tumor, Ki67, which is a marker
of odontogenic cysts and tumors, especially in prea- associated with cell proliferation, was detected. In both
meloblasts and secretory ameloblasts [27]. In both the our case and in previous studies [2, 4], the expression of
previously presented and current POT cases, CK19 Ki67 was low (< 2%) in both epithelial and mesenchymal
predominated in cubic and columnar epithelial cells cells, which is similar to other benign odontogenic tumors,
[2, 9], suggesting that these epithelial linings express such as OMs [31].
diverse degrees of maturation. This can be supporting We herein report the first case of POT in South Korea
evidence that the tumor originated from primordial since it was newly categorized in the latest WHO classifi-
cellular components of the enamel organ [4]. Vimen- cation in 2017. As there are only 16 documented cases, in-
tin was variably positive in cells of mesenchymal cluding this report, in English literature worldwide, more

Table 3 Immunohistochemistry results of the previous cases and current case

Position Antibody Immunohistochemistry results Positivea (%)
Epithelial cells CK19 Positive (mainly in columnar epithelium) 10/10 (100%)
Vimentin Positive 9/9 (100%)
Mesenchymal tumor cells Vimentin Positive 9/9 (100%)
α-SMA Negative 0/9 (0%)
S-100 protein Negative 0/9 (0%)
a
.10 cases were analyzed for CK19 using immunohistochemical analysis, and all cases showed positive results in the tumor epithelium. Vimentin was also 100%
positive throughout the tumor in all nine cases in which it was analyzed. α-SMA and S-100 protein were negative in all nine cases
Sun et al. Diagnostic Pathology (2019) 14:92 Page 7 of 8

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