Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 32 (2020) 285–290

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Journal of Oral and Maxillofacial Surgery, Medicine, and

Pathology
journal homepage: www.elsevier.com/locate/jomsmp

Case Report

Pediatric cemento-ossifying fibroma of the anterior mandible: A case report T

a, a,b a c a
Hitoshi Miyashita *, Hikari Suzuki , Keiko Matsui , Naoko Sato , Jun Kitamura ,
Hiroyuki Kumamotod, Tetsu Takahashia
a
Division of Oral and Maxillofacial Surgery, Department of Oral Medicine and Surgery, Graduate School of Dentistry, Tohoku University, 4-1 Seiryo, Aoba, Sendai, 980-
8575, Japan
b
Department of Dentistry and Oral Surgery, Sendai Medical Center, 2-11-12 Miyagino, Miyagino, Sendai, 983-8520, Japan
c
Maxillofacial Prosthetics Clinic, Tohoku University Hospital, 4-1 Seiryo, Aoba, 980-8575, Sendai, Japan
d
Division of Oral Pathology, Department of Oral Medicine and Surgery, Graduate School of Dentistry, Tohoku University, 4-1 Seiryo, Aoba, Sendai, 980-8575, Japan

A R T I C LE I N FO A B S T R A C T

Keywords: Cemento-ossifying fibroma (COF) is a clinicopatological variant of ossifying fibroma which is benign fibro-
Cemento-ossifying fibroma osseous neoplasm affecting jaw or cranial skeletons. It is also called central ossifying fibroma. COF is a slow-
Fibro-osseous lesion growing tumor and occurs exclusively in the tooth bearing areas of the jaws. This tumor particularly affects adult
Odontogenic tumor female and rarely occurs in children. Here we show a rare case of COF involving the anterior mandible in an 8-
Mandible
year-old girl. She was referred to our department with painless and hard swelling of the mandibular symphysis.
Child
Computed tomography (CT) showed that well circumscribed and mainly radiodense lesion with radiolucent
areas expanded the inferior border of the mandible. The size of this lesion is 35 × 30 × 24 mm. Pathological
diagnosis was COF. She had underwent two times of surgery for tumor enucleation through an intraoral ap-
proach, and after that, we removed bilateral central incisors with residual tumor around the roots. There has
been no sign of recurrence 1 years after the last surgery. We have continued follow-up observation.

1. Introduction region in an 8-year-old girl.

According to the WHO classification of head and neck tumors 2017,
cemento-ossifying fibroma (COF) is classified into fibro-osseous lesions,
and is also categorized into odontogenic tumor [1]. This tumor mainly
affects adult female, and pediatric COF affecting jaws is extremely rare.
It occurs exclusively in the tooth bearing areas of the mandibular pre-
molar and molar area [1–5].
Radiographic examination almost commonly shows a well-defined
radiolucent area containing radiopaque material, and this tumor
sometimes surrounded by a sclerotic border [1,6].
Histopathologically, this type of tumor is derived from the me-
senchymal blast cells of the periodontal ligament, and have a potential
to form fibrous tissue, cement and bone or a combination of such ele-
ments [1,7,8]. Eversole et al. has reported that these cementum-like
structures are associated with membranous bone [9].
The first choice of treatment is conservative surgery with long-term
follow-up [1,2,6]. It has recently reported that the range of recurrence
rate of ossifying fibroma (OF) were from 12 % to 28 % [7,9,10].
As far as we investigated, there have been no case with COF in-
volving the anterior mandible in children under the age of 10. The
following report presented a rare case of COF of anterior mandibular
2. Case report
An 8-year-old girl was referred to our department for examination
of painless swelling of the mandibular symphysis. The swelling has
seemed to be gradually growing for 1 year since she had been noted to
have the localized lesion. She had facial asymmetry, and there was a
palpable bony hard swelling without redness or infection. There was no
trismus, numbness, toothache, past history of facial trauma or previous
similar swelling (Fig. 1A and B).
Panoramic radiography showed well-defined, expansive and radio-
dense lesion with radiolucent areas of the anterior mandible. This lesion
caused root resorption of right lateral incisor and displaced some lower
teeth (Fig. 2). Computed tomography (CT) revealed that the size of this
lesion was 35 × 30 × 24 mm. The lesion expanded inferior border of
the mandible with thinning of cortical bone, but did not involve the
right mental foreman. The internal structure was mainly radiopaque
with diffuse radiolucent areas (Fig. 3A, B and C). 99mTc-methylene
diphosphonate (MDP) bone scan demonstrated the intense radiotracer
uptake in this lesion (Fig. 4).
Pathological diagnosis was COF. Enucleation of the mandibular

⁎
Corresponding author.
E-mail address: miyashita@dent.tohoku.ac.jp (H. Miyashita).

https://doi.org/10.1016/j.ajoms.2020.03.007
Received 3 February 2020; Received in revised form 12 March 2020; Accepted 22 March 2020
Available online 18 May 2020
2212-5558/ © 2020 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd All rights reserved.
H. Miyashita, et al.
Fig. 1. Facial expression and intraoral findings. (A) Facial asymmetry with
bulge of the mandibular symphysis region. (B) Intraoral swelling of the anterior
mandible crossing the midline.
tumor with tooth extraction of the right lateral incisor through an in-
traoral approach was performed (Fig. 5A). The tumor had caused dis-
placement of inferior alveolar nerve of the right side. After 10 months
from primary surgery, secondary tumor enucleation with removing
right canine and first premolar teeth was performed. In addition, after 2
years has passed since the secondary operation, we removed bilateral
Fig. 2. Panoramic radiography. Well-defined and radiodense lesion with some radiolucent areas causing root resorption of the right lateral incisor and displacement
some teeth in the anterior mandibular region.
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Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 32 (2020) 285–290
central incisors with residual tumor around the roots. Histopathological
examination revealed hypercellular fibroblastic stroma containing bone
trabeculae and irregularly shaped cementum-like tissue. Osteoblastic
rimming of the bone trabeculae was also seen (Fig. 5B and C). There has
been no sign of recurrence 1 year after the last surgery (Fig. 6A and B),
and she has a removable denture (Fig. 6C). Postoperative imaging
shows bone formation of the anterior mandible (Fig. 7A and B). Long-
term follow-up observation has been needed, and we have planned
occlusal reconstruction.
3. Discussion
COF is classified into fibro-osseous and osteochondromatous lesion,
and is also categorized into benign mesenchymal odontogenic tumors.
COF is one of the three clinicopathological variants of OF, which have
been identified as COF, juvenile trabecular ossifying fibroma (JTOF)
and juvenile psammomatoid ossifying fibroma (JPOF). The synonyms
for COF are central OF, cementifying fibroma and periodontoma [1].
Epidemiology, it has proposed that the peak incidence of COF has been
in the third and fourth decades of life. Furthermore, there is a definite
female prediction with a ratio as high as 5:1 [1,9,12]. COF occurs ex-
clusively and more commonly in the tooth-bearing area of the mandible
rather than that of maxilla, and the most common site is mandibular
premolar and molar area [1–5]. Some authors proposed that the COF
occurring at maxilla behaves more aggressively with more obvious
symptoms and signs than those in mandible [13,14]. It may result from
the different physiological natures between mandible and maxilla. The
mandible contains a thicker outer cortex with loose inner marrow, and
the thick cortex is a barrier for the growth and expansion of the lesion.
On the contrary, the lesion in maxilla is easier to expand in thin cortex
and inner cancellous bone [14]. In this report, in an 8-year-old girl, the
lesion occurred in the mandibular symphysis and mainly involved the
anterior mandibular area. To our knowledge, there is no case with
pediatric COF involving the anterior mandible under the age of 10.
The clinical features vary depends on cases. Generally, COF presents
as a painless expansion, and large tumor expands maxillary sinus or
inferior border of the mandible. Radiographically, it appears typically
as a radiolucent lesion in an early stage. However, over time, tumor
becomes progressively more radiopaque [1,15]. According to Guan-
seelan et al, the most common finding was well-defined radiolucent
lesion and jaw bone expansion with or without sclerotic border, and
mixed radiolucent and radiopaque components was the second most
common features [16]. In our case, the patient had painless and bony
hard swelling of the mandibular symphysis without numbness, which
led to facial asymmetry. The images showed that the lesion was well
H. Miyashita, et al. Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 32 (2020) 285–290

Fig. 4. 99mTc-MDP. The radioactive concentration at the site of the anterior

mandible.

circumscribed and had predominantly radiopaque components with

Fig. 3. CT and 3D-CT scans. Well-circumscribed and radiodense lesion with
radiolucent areas (35 × 30 × 24 mm) expanding the inferior border of the
mandible with thinning of cortical bone.
surrounding radiolucent area. The expansion of buccal, lingual and
inferior border cortex with teeth displacement were also identified as
previously reported [15,17]. Furthermore, 99mTc-MDP revealed the
radioactive concentration in this lesion. Because of these findings and
noticing the lesion before 1 year, it was indicated that the tumor had
tended to mature for a long time. Hitopathologically, the tumor of this
case was composed of hypercellular fibroblastic stroma containing a lot
of irregularly shaped calcified structures and bone trabeculae. Then, the
pathological findings of resected tumor was mostly similar to that of
JTOF, and partially to that of JPOF. It has previously reported that in
childhood, the lesion has a possibility to present as juvenile aggressive
COF, which is clinically more aggressive with high vascularity [18].
While the etiology has yet been unknown, trauma may act as a pre-
disposing factor, which suggest a connective tissue-reactive etiology
rather than a neoplastic one [19,20]. It has been proposed that trauma
or dental extractions may leave part of the periodontal membrane at-
tached to the wall of the alveolus, predisposing to stimulation and
subsequent deposition of cementum; thus, supporting the theory of
periodontal membrane as the origin of COF [7,20]. In this case, al-
though the patient was 8-year-old, the growth rate of this tumor was
clinically slow, and the vascularity was histopathologically low. So this
case had no finding to reveal juvenile aggressive COF. In addition, there
was no history of trauma and injury of facial region.
The differential diagnosis for COF has been broad: 1) Cemento-
osseous dysplasia (COD) is a non-neoplastic fibro-osseous lesion of the
tooth-bearing regions of the jaws. Radiolucent, mixed density or
radiopaque lesions of the apex of vital teeth has pointed to COD. COD is
sub-classified into three variants: periapical COD, when affected areas
are localized to the periapical regions of the mandibular anterior teeth;
focal COD is associated with a single tooth; and florid COD has multi-
quadrant lesions [1,21,22]. These characteristics are inappropriate for
this case. Furthermore, the lesion of our case was well defined, could be
shelled out from surrounding tissue, and submitted as large fragments.
This characteristic of “shelling out” and gross appearance are quite
different from that of COD, with its gritty fragmented nature [1,21,22].
Histologically, COF is characterized by osteoblastic rimming as our case
shows, and it has been considered a hallmark of the microscopic pre-
sentation. On the other hand, osteoblastic rimming is generally rare in
COD, or it is found only focally [1,21,22]. 2) Osteoblastoma is a benign
bone-forming tumor with prominent osteoblastic rimming. Some of the
bone in this lesion are strongly haematoxophilic, resulting in the
characteristic of blue-bone appearance [1]. These features were not

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Fig. 5. Tumor tissue and histopathological examination. (A) Resected spe-

cimen. (B) and (C) Bone trabeculae and cementum-like tissue in hypercellular
fibroblastic stroma. (hematoxylin and eosin stain, ×80).

found in this case. 3) Then, osteoclast-type giant cells as shown in giant

cell lesions was not appeared. Since our case showed well-demarcated
fibro-osseous lesion composed of increased cellular fibrous tissue as-
sociated with bone- and cementum-like hard tissue formation and the
characteristic of “shelling out”, it was not so difficult to diagnose the
lesion as COF.
The first choice for the treatment of COF has recently been indicated
conservative surgery with long-term follow-up because this lesion can
be excised with no recurrence in most cases [1,2,6]. Some authors have
reported that expansive recurrent lesion after the surgery and untreated
massive tumors may require en bloc resection [1,17]. The recurrence
rate of OF were from 12 % to 28 % [7,9,10], but in COF alone, it has
been unclear for its rarity. Based on these findings and knowledge, we
selected this treatment strategy with an intraoral approach, as a mini-
mally invasive surgery. The management of tooth involved in this kind
of lesion has also been controversial. As a result, we finally needed to
remove five teeth in this case.
Regarding genetic profile, some studies assessed mutations in the
Fig. 6. Postoperative facial expression and intraoral findings. (A) Facial sym-
metry without paresthesia of inferior alveolar nerve. (B) Missing five lower
teeth. (C) With a removable partial denture.
CDC73 (HRPT2) tumor suppressor gene which encodes the parafi-
bromin protein [23,24]. However, even though somatic mutations were
found in OF cases, almost all of them expressed normal parafibromin.
So de Mesquita Netto AC et al. have concluded that the contribution of
HRPT2 inactivation to the pathogenesis of OF is marginal at best and is
limited to progression [24]. This gene seems to have a more significant
role in OF related to hyperparathyroidism–jaw tumor syndrome
[24,25]. In 2018, Pereira TDSF et al. first described that deregulation of
key Wnt/β-catenin signaling pathway genes appeared to be relevant to

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Fig. 7. Postoperative (A) panoramic radiography and (B) 3D-CT scan. No signs of recurrence in the mandible, and showing bone formation.
the molecular pathogenesis of COF [11].
As points to be aware of regarding COF, although the malignant
transformation had not been described until recently, in 2015, Lee YB
et al. reported a case of low-grade osteosarcoma arising from COF [26].
Post operative follow-up with long-term is mandatory.
Ethical approval
Written informed consent was obtained from the patient and her
parents.
Declaration of Competing Interest
Nothing to disclose.
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