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Int. J. Oral Maxillofac. Surg.

2016; 45: 368376


http://dx.doi.org/10.1016/j.ijom.2015.12.004, available online at http://www.sciencedirect.com

Clinical Paper
Clinical Pathology

Juvenile ossifying fibroma of the


jaw: a retrospective study of 15
cases
J. Han, L. Hu, C. Zhang, X. Yang, Z. Tian, Y. Wang, L. Zhu, C. Yang, J. Sun, C.
Zhang, J. Li, L. Xu: Juvenile ossifying fibroma of the jaw: a retrospective study of 15
cases. Int. J. Oral Maxillofac. Surg. 2016; 45: 368376. # 2015 International
Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights
reserved.

Abstract. The management of patients with juvenile ossifying fibroma (JOF) remains
controversial. To explore the correlations between different treatments and the
patient prognosis, 15 cases of JOF of the jaw were reviewed. Five patients were
male and 10 were female. Patient age at the time of disease onset ranged from 7 to
18 years (mean 10.9 years). Nine tumours were located in mandible and six in the
maxilla. These cases typically manifested clinically as painless swelling of the jaw
(9/15, 60%); 40% (6/15) of the cases were associated with pain, diplopia, stuffy
nose, and/or rapid growth. Images of JOF can show a radiolucent, mixed, or ground
glass-like appearance. Pathological examinations revealed 10 cases of juvenile
trabecular ossifying fibroma (JTOF) and five cases of juvenile psammomatoid
ossifying fibroma (JPOF). In terms of the treatment plan, six patients initially
received radical surgery; nine patients underwent conservative treatment, among
whom six (6/9, 66.7%) had one or more recurrence. At the end of the follow-up
period, 12 patients had no evidence of tumour recurrence and three cases were alive
with a tumour. In summary, surgeons should develop the surgical plan according to
the extent of the lesion, relapse status, growth rate, and family choice, and these
patients should be followed up closely.

Ossifying fibroma (OF) is a type of benign


fibro-osseous lesion. This tumour is characterized by clear boundaries and cell-rich
fibrosis, and contains varying amounts of
calcified tissue resembling bone, cementum, or both. OF lesions are classified as
conventional ossifying fibroma and juvenile ossifying fibroma (JOF).1 Conventional OF is mainly seen in adults. JOF
usually occurs in children or adolescents,
0901-5027/030368 + 09

and it is therefore also known as juvenile


ossifying
fibroma
active/aggressive
(JAOF). In 2005, the World Health Organization (WHO) noted that the age at onset
of JOF is 15 years and younger.1 However,
JOF has also been documented in adults.2,3
JOF has characteristics of aggressive
growth, is associated with damage to the
cortical bone, and may involve the nasal
cavity, eyes, and even cerebrum. Howev-

J. Han1,5, L. Hu1,5, C. Zhang2,


X. Yang1, Z. Tian2, Y. Wang1, L. Zhu3,
C. Yang4, J. Sun1, C. Zhang1, J. Li2,
L. Xu1
1
Department of Oral and MaxillofacialHead
and Neck Oncology, Ninth Peoples Hospital,
Shanghai Jiao Tong University School of
Medicine, Shanghai, China; 2Department of
Oral Pathology, Ninth Peoples Hospital,
Shanghai Jiao Tong University School of
Medicine, Shanghai, China; 3Department of
Radiology, Ninth Peoples Hospital, Shanghai
Jiao Tong University School of Medicine,
Shanghai, China; 4Department of Oral
Surgery, Ninth Peoples Hospital, Shanghai
Jiao Tong University School of Medicine,
Shanghai, China

Key words: jaw; JOF; JPOF; JTOF; treatment.


Accepted for publication 7 December 2015
Available online 28 December 2015

er, there are no reports of cases of malignant transformation or metastasis. The


imaging characteristics of JOF show expansive, well-defined radiolucent or
mixed images that are separated from
the surrounding normal bone.4 Based on
histopathology, JOF is divided into the
5
These authors contributed equally to this
work.

# 2015 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Juvenile ossifying fibroma of the jaw


following two subtypes: juvenile psammomatoid ossifying fibroma (JPOF) and juvenile trabecular ossifying fibroma (JTOF). In
addition, JOF usually contains multinucleated giant cells, whereas conventional OF
does not.5,6 Conventional OF usually presents as a slow-growing mass of bone expansion that is usually without symptoms
and rarely recurs; conversely, 38.5% of JOF
cases are associated with mandibular swelling and pain and there may be short-term
rapid growth.7 Therefore, surgeons should
consider clinical features, radiological
characteristics, and pathological features
when diagnosing JOF. Key points to be
considered include age, status of growth,
and histopathology.8
The management of patients with JOF
remains controversial. According to the
literature, the recurrence rate after surgery
is approximately 3058%.1,3,911 Therefore, designing an appropriate treatment
plan for the JOF patient could help to
improve the prognosis and quality of life.
Depending on biological behaviour and
the lesions involved, treatment can be

conservative or radical.12 Some researchers have emphasized the aggressive


growth characteristics and high recurrence
rate of JOF, leading them to advocate local
radical surgery.4,13,14 In contrast, others
consider that conservative treatment is
more beneficial for young patients when
taking into account their growth and development, their appearance, and the preservation of chewing and nerve
functions.12,15,16
This study was performed to review the
clinical features, imaging features, and
pathological characteristics of 15 cases
of JOF treated at the authors institution.
The relationships between the different
treatment options and the prognosis are
discussed and the most suitable treatments
for JOF are explored.
Materials and methods

A total of 15 patients with JOF treated


between 2005 and 2014 in the Department
of Oral and MaxillofacialHead and Neck
Oncology of the study institution were

369

included. Each patients medical history


was reviewed carefully to collect clinical
data (including age, sex, disease location,
symptoms, surgical approach, and prognosis), radiological features, histological
type, and other information.
According to the anatomical location,
the mandible was divided into anterior
(left canine to right canine), posterior (first
premolar to third molar), angle, ramus,
and condyle. The maxilla was divided into
anterior (left canine to right canine) and
posterior (first premolar to the maxillary
tuberosity). Imaging data were used to
analyze the lesion and surrounding tissue
boundaries and the internal radiographic
architecture. The internal radiographic architecture was divided into mixed radiopaque and radiolucent, unilocular or
multilocular radiolucency, and ground
glass opaque. An oral pathology specialist
classified the histological subtype as either
JTOF or JPOF based on the 2005 WHO
classification criteria for head and neck
cancer.1 Surgeries performed included
conservative treatments (curettage and

Table 1. Clinical information, treatment, and follow-up information for the 15 cases of juvenile ossifying fibroma (JOF).
Patient
No./sex

Age at
onset
(years)

Age at
operation
(years)

Conservative treatment
1/M
8
8

Size
4.5  4

Symptoms

Right maxilla

Curettage

Left maxilla
(posterior)
Right mandible
(posterior)
Left maxilla

Curettage

14

19

2  1.5

Swelling

3/M

13

13

54

Swelling

4(R)/M

18

20

32

Swelling, pain

5(R)/F

16

64

Swelling

6(R)/F

12

15

54

Swelling

7(R)/M

15

17

98

8(R)/M

10

12

10  7

9(R)/F

19

98

Swelling,
rapid growth
Swelling,
rapid growth
Swelling,
stuffy nose,
diplopia

19

12  8

Swelling

64

Swelling

11/F

11

11

12/F

6  4.5

Swelling

13/F

32

Swelling

14/F

4  3.5

15/F

11

15

Swelling, pain,
diplopia,
rapid growth
Swelling, pain,
rapid growth

64

Surgery

Swelling

2/F

Radical treatment
10/F
13

Location

F, female; M, male; NED, no evidence of disease; (R), recurrence.

Enucleation

72; alive
with tumour
20; alive
with tumour
38; NED

Right mandible
(posterior)
Left mandible
(posterior/angle)
Right mandible
(posterior)
Left mandible

Curettage/resection + fibula

124; alive
with tumour
95; NED

Curettage/resection + iliac crest

146; NED

Curettage/resection + fibula

105; NED

Curettage/resection + fibula

40; NED

Right maxilla

Curettage/resection + fibula

128; NED

Left mandible
(posterior)
Right mandible
(posterior)
Left mandible
(posterior/angle)
Right mandible
(condyle)
Left maxilla
(posterior)

Resection + fibula

87; NED

Resection + iliac crest

58; NED

Resection + iliac crest

36; NED

Resection + costal cartilage

15; NED

Resection

18; NED

Resection

13; NED

Right maxilla

Curettage

Follow-up
(months);
outcome

370

Han et al.

Table 2. Radiographic features and pathology of 15 cases of juvenile ossifying fibroma (JOF).
Pathology
JTOF

JPOF

Patient No.
4
6
7
11
1
12
8*
2
13
14*
10
3
5
9
15

Radiographic features
Ground glass opaque, well-defined border
Mixed radiopaque and radiolucent, well-defined
Multilocular radiolucency, well-defined border
Mixed radiopaque and radiolucent, well-defined
Mixed radiopaque and radiolucent, well-defined
Unilocular radiolucency, well-defined border
Multilocular radiolucency, well-defined border
Not available
Mixed radiopaque and radiolucent, well-defined
Not available

border
border
border

border

Ground glass opaque with central radiolucency, well-defined border


Mixed radiopaque and radiolucent, well-defined border
Ground glass opaque with central radiolucency, well-defined border
Multilocular radiolucency with ground glass opaque, well-defined border
Ground glass opaque with central radiolucency, well-defined border

JTOF, juvenile trabecular ossifying fibroma; JPOF, juvenile psammomatoid ossifying fibroma.
*
JTOF with aneurysmal bone cyst.

enucleation) and radical surgery (complete resection with or without reconstruction).


Due to the retrospective nature of this
study, it was granted an exemption from
requiring ethics approval in writing by the
institutional review board of the study
hospital.

Results
Clinical data

Of the 15 cases of JOF, five were male and


10 were female, giving a male to female
ratio of 1:2. The age at onset ranged from 7
to 18 years (mean 10.9 years). The ages of
JTOF patients ranged from 9 to 13 years
(mean 11 years) and the ages of JPOF
patients ranged from 7 to 18 years (mean
10.9 years). The disease duration ranged

from 2 weeks to 5 years, with an average


of 18.2 months (Table 1).
The lesions were located in the mandible
in nine cases and in the maxilla in six cases,
with three involving the maxillary sinus. In
mandibular cases, the site was most often
posterior (5/9, 55.6%), followed by a combination of posterior and angle (2/9,
22.2%). For the upper jaw, unilateral involvement of the entire maxilla (4/6,
66.7%) was most common, followed by
the posterior of the maxilla (2/6, 33.3%).
Sixty percent (9/15) of cases manifested
clinically as a slowly growing painless
mass in the jaw. Forty percent of cases
(6/15; four cases involving the maxilla and
two involving the mandible) presented
with pain, diplopia, stuffy nose, and/or
rapid growth. Clinical examination
revealed an immobile hard mass, and
egg shell crackling was occasionally noted
when a cystic lesion developed.

Before surgery, most patients had a


diagnosis of ossifying fibroma (10/15,
66.7%). Other diagnoses included ameloblastoma (n = 2, 13.3%), fibrous dysplasia
(n = 1, 6.7%), chondromyxoid fibroma
(n = 1, 6.7%) and aneurysmal bone cyst
(n = 1, 6.7%).
Imaging features

Images of all patients showed a mass with


clear boundaries. There was generally no
soft tissue involvement. Computed tomography (CT) and/or panoramic images were
available for a total of 13 patients. According to the component and proportion of
soft tissue and hard tissue in the internal
structure, the tumour masses presented as
mixed radiopaque and radiolucent (5/13,
38.5%), unilocular or multilocular radiolucency (3/13, 23.1%), and ground glass
opaque (4/13, 30.8%) (Table 2). Three of

Fig. 1. Imaging findings in juvenile ossifying fibroma (JOF): case 15. (A) Preoperative axial CT showing a bulging mass in the maxilla, a ground
glass-like appearance, and central cystic changes. (B) Preoperative sagittal CT showing a mass intruding into the maxillary sinus and root
displacement. There is a high-density shadow in the surrounding area.

Juvenile ossifying fibroma of the jaw

371

Fig. 2. Histopathological features of juvenile ossifying fibroma (JOF). (A) Juvenile trabecular ossifying fibroma (JTOF) (case 4) has a cell-rich
fibrous tissue background with cell-containing immature trabecular bone. A layer of hypertrophic osteoblasts, which form a grid-like structure,
surrounds the trabecular bone. (B) Juvenile psammomatoid ossifying fibroma (JPOF) (case 15) has a fibrous stroma containing spherical
mineralized bodies. The bodies are like cementum, with basophilic cores and an eosinophilic exterior. (C) Aneurysmal bone cyst formed within
JTOF (case 8). The capsule is filled with red blood cells.

the ground glass opaque cases were associated with central radiolucent regions
(Fig. 1), and one case had multiple radiolucent regions associated with ground
glass plaques. CT imaging and three-dimensional reconstruction of the different
orientations showed cortical bone destruction and absorption in nine cases (9/13,
69.2%), with adjacent tooth or root displacement in seven (7/13, 53.8%).

one underwent local curettage. Cases 4, 5,


6, and 7 had recurrence after 8 months, 2
years, 6 months, and 11 months, respectively. Case 8 had local recurrences after
twice undergoing curettage, at 6 months
and 7 months after the procedure. Case 9
had recurrences at 5 years and 15 months
after surgeries. Overall, recurrence oc-

curred at between 6 months and 5 years,


with an average of 17.1 months. Six
patients initially had local radical surgery
and none had a recurrence.
Nine patients underwent reconstructive
surgery. One patient with JOF in the maxilla underwent a fibula muscle flap transplantation (Fig. 3). Of the eight mandible

Histopathological features

General examination revealed that JOF


was usually a grey mass with a clear
boundary. Tumour sizes ranged from 2
to 12 cm, with an average of 6.03 cm.
Those with diameters 5 cm accounted
for 66.7% (10/15) of cases. Dissection of
the tissue showed gravel-like or hard tissue. Of the 15 cases of JOF, 10 were JTOF
and five were JPOF (Table 2). Under the
microscope, JTOF demonstrated a cellrich fibrous tissue background with a strip
of osteoid-containing cells or immature
trabecular cells. The trabecular bone was
surrounded by a layer of hypertrophic
osteoblasts, which together formed a
grid-shaped region. Some regions showed
aggregations of osteoclast-like multinucleated giant cells. Twenty percent (2/
10) of cases were associated with an aneurysmal bone cyst (Fig. 2A, C). JPOF
was characterized by fibroblastic stroma
and basophils containing sand-like structures that resembled cementum (Fig. 2B).
Types of surgery and prognosis

All patients underwent surgical treatment,


including conservative treatment and local
radical surgery. Nine patients first underwent conservative treatment, among
whom six had one or more recurrence,
giving a recurrence rate of 66.7% (6/9).
Among those experiencing a recurrence,
five underwent local radical surgery and

Fig. 3. Imaging findings in juvenile ossifying fibroma (JOF): case 9. (A) The lesion recurred
after multiple maxillary curettages. Axial CT showing multiple transparent shadows accompanied by ground glass-like plaques. (B) CT reconstruction revealing lesions involving the nasal
cavity and orbital cavity, as well as cortical discontinuity. (C) Immediate CT three-dimensional
reconstruction after maxillary expansive resection and fibula muscle flap transplantation
showing ideal recovery of the jaw morphology. (D) Axial CT showing a metal plate of the
correct shape without evidence of recurrence at 1 year and 2 months after surgery.

372

Han et al.

cases, three underwent a free iliac crest


graft (Fig. 4), one case with condyle involvement had a costal cartilage transplantation (Fig. 5), and four cases
underwent fibula flap transplantation
(Fig. 6).
The 15 patients were followed up for
13146 months, with an average followup of 66.3 months. The follow-up of
patients with multiple surgeries was
recorded from the date of the initial surgery (Table 1). All patients were alive at
the end of follow-up: 12 patients had no
evidence of disease (tumour-free survival)
and three patients were living with
tumours.
Discussion

JOF is a variant of OF that occurs mainly


in the cranial and facial bones of young
patients. JOF can lead to severe morphological and functional defects. JOF and
other benign fibrous lesions have great
similarities in terms of their clinical features, imaging characteristics, and pathology.
Therefore,
a
systematic
understanding of the disease can enable
clinicians to perform an accurate diagnosis, develop a reasonable treatment plan,
and predict the prognosis. This study retrospectively analyzed 15 cases of JOF
with regards to their clinical, radiological,
and pathological features and analyzed the
prognoses associated with the different
treatment options. The aim was to develop
more appropriate treatment methods. This
study reports on JOF in a relatively large
cohort of Chinese patients.
OF can occur at any age, but is mainly
seen in adults aged 3040 years. JOF
patients are relatively young. In the
2005 WHO definition, the age at onset
of JOF is 15 years. However, according
to a report by Johnson et al., the age at
onset of JOF ranges from 3 months to 72
years.3 Studies reported in the literature
have indicated that the average age at
onset of the two JOF subtypes differs:
the age at JPOF onset is 10 to 25 years,10
whereas JTOF patients are relatively
younger, at 8.512 years.4 The ages of
the patients with the two subtypes in the
present study were similarly young: the
average age of JTOF patients was 11 years
and the average age of JPOF patients was
10.9 years. In terms of the sex distribution,
various case reports in the literature have
shown a slight difference.3,5 In the present
group of patients, JOF was more commonly seen in females, with a male to female
ratio of 1:2.
JTOF usually occurs in the jaw,4 whereas JPOF occurs mainly in the paranasal

Fig. 4. Imaging findings in juvenile ossifying fibroma (JOF): case 11. (A) Preoperative
panoramic radiograph showing a bulging body in the right mandible. (B) Four years after
mandibular segmental resection and iliac bone graft, the mandible is in good shape with no
evidence of recurrence.

sinus bone wall.3,17 There are a few case


reports of JPOF in the jaw.2,18 In this
study, the lesions in the 15 patients with
JOF (10 cases of JTOF and five cases of
JPOF) all occurred in the jaw. Different
reports have shown different sites of disease onset.5,19,20 In this study, there were
slightly more patients with disease in the
lower jaw (nine cases) than in the upper
jaw (six cases).
Locally aggressive growth is an important clinical feature that differentiates JOF
from conventional OF. It has been
reported that 38.5% of JOF patients have
swelling with pain,7 while conventional
OF is associated with painless swelling in
the jaw. When the tumour is located in the
upper jaw and becomes large, it can involve the surrounding tissues such as the
eyes17,21 and nasal cavity,22 thereby
resulting in exophthalmos, nasal congestion, and other symptoms. In the group of
patients presented here, 40% (6/15; four
cases involving the maxilla and two involving the mandible) had pain, diplopia,
stuffy nose, and/or rapid tumour growth.
The imaging characteristics of this
group of JOF patients were found to be
similar to those reported in the literature.4,23 According to the internal components of hard and soft tissue, tumours can
present as radiolucent, mixed, opaque, and
ground glass-like. There is a high-density

boundary between the lesions and the


surrounding normal bone. The imaging
characteristics are often associated with
histological types.23 JPOF usually resembles ground glass and JTOF usually looks
like mixed or radiolucent shadows. This
group of cases had similar features. Pathologies that need to be distinguished
from JOF using imaging studies include
fibrous dysplasia, osseous dysplasia,
odontoma, and ameloblastoma. Although
fibrous dysplasia can resemble ground
glass, it usually has unclear boundaries
and may involve multiple bones. Osseous
dysplasia usually presents as calcification
in the space between the roots or as a
cotton-like calcification involving the bilateral upper and lower jaws. Odontoma
and ameloblastoma are also common in
younger patients, although odontoma usually presents as a mass with mixed densities around the roots, with clear
boundaries and visible non-erupted teeth
or shadows of teeth of varying sizes.
Ameloblastoma usually presents as multiple radiolucent regions with discrete, corticated borders. Aneurysmal bone cyst
associated with JOF is reported widely,3,4,10,24,25 and this is more common in
young patients and shows levelled fluid
under CT examination.10 In this study,
there were two cases of JTOF with aneurysmal bone cyst. These two patients were

Juvenile ossifying fibroma of the jaw

373

Fig. 5. Imaging findings in juvenile ossifying fibroma (JOF): case 13. (A) Preoperative panoramic radiograph showing a transparent shadow in the
right condylar region, with clear boundaries. (B) Preoperative coronal CT showing shadows of mixed densities in the right condylar region. (C) Six
months after condylar tumour resection and cartilage repair, the shape of a metal plate is partially seen, and there is no evidence of recurrence. (D)
Postoperative CT reconstruction showing good recovery of jaw shape.

10 years and 7 years old at onset, which is


consistent with findings in the literature.
Conventional OF lesions usually have
limited regions and clear boundaries.
Patients usually undergo surgical excision
of the tumour. Recurrence is rare and the
prognosis is good. However, there is currently no consensus on the treatment plan
for JOF. Clinical treatments can be divided into conservative and radical treatments, and the histological subtype has
no effect on treatment options. Considering the aggressive growth pattern and high
recurrence rate of JOF, some researchers
advocate local radical surgery to
completely remove as much of the involved tissue as possible while preserving
the important surrounding structures. Nevertheless, excessive removal of the surrounding normal bone tissue is not
recommended.4,13,14 Conservative treatments including curettage, enucleation,
and partial resection, aim to limit facial
deformity and dysfunction and preserve
growth and development, a normal appearance, and chewing and nerve functions in young patients.12,15,16 When

aggressive growth is obvious, which is


marked by rapid growth, cortical thinning
or perforation, and the displacement of
teeth or root resorption, the patient should
undergo complete resection.12
In a recent study, researchers found that
the simultaneous amplification of MDM2
(E3 ubiquitin-protein ligase gene) and
RASAL1 (RAS protein activator like 1
gene) was significantly higher in juvenile
ossifying fibromas compared with nonjuvenile ossifying fibromas. Moreover,
among the nine cases of JOF with
MDM2 and RASAL1 amplification, three
demonstrated local invasiveness or rapid
growth and four were recurrent. This abnormality is not only a potential molecular
diagnostic marker, but may also be indicative of aggressive forms of the disease
with a higher risk of recurrence,26 and
therefore may indicate a more radical
treatment plan.
As JOF patients are relatively young,
clinicians should aim to maintain or restore the patients appearance during treatment to reduce the impact on their growth
and development. Individualized treat-

ment plans were developed for each patient in the present study, after taking
various factors into account. These factors
included the site of the lesion, involvement of the surrounding vital tissue, patient age, growth rate, and parental choice.
Of the 15 patients, nine initially underwent
conservative treatment and six received
local radical surgery.
In cases of large or rapidly growing
lesions, among others, complete resection should be chosen as the initial surgery. Additionally, due to the anatomical
structure, maxillary lesions often involve
the nose, eyes, skull, and other tissues;
this makes complete resection difficult.
Meanwhile, with relatively less important structures around mandibular
lesions, segmental resection is not always necessary. Take case 3 for example: the lesion had a limited range, there
were clear boundaries between the lesion
and the surrounding normal bone, and
the jawbone cortex had continuous
edges; in this case enucleation of the
tumour achieved a good prognosis
(Fig. 7).

374

Han et al.

Fig. 6. Imaging findings in juvenile ossifying fibroma (JOF): case 8. (A) The mandibular lesion relapsed after curettage. Panoramic radiograph
showing a mixed shadow containing incomplete permanent crowns. (B) The lesion expanded 7 months after a second curettage. (C) Sagittal CT
showing transparent shadows with mixed densities. (D) Axial CT showing significant bulging in the lingual mandibular cheek. (E) CT
reconstruction revealing severe bone destruction and cortical discontinuity. (F) Two years and 1 month after mandibular segment resection and
fibula flap transplantation, there was no evidence of recurrence.

According to reports published in the


literature, the recurrence rate after surgery
is approximately 3058%.1,3,911 The total
recurrence rate calculated from the present
data is 40% (6/15), with the rate after
conservative treatment being high at
66.7% (6/9) and the rate following radical
surgery being zero. Since malignancies
have not been reported, these neoplasms
can safely be left when they are in a stable
state. In this study, three patients were
alive with the tumour at the end of follow-up, and the tumour did not show
obvious enlargement.

Defect reconstruction can be carried


out at the time of excision 13,2729or in a
second-stage procedure.30 There are
reports of no repair or of immediate
repair using a reconstruction plate to
restore the appearance after resection
of JOF.18,31 In this study, a total of nine
patients underwent immediate reconstruction after resection. Based on the
site and size of the defect, three methods
of restoration were used, including
vascularized fibula flap transplantation
(five cases), free iliac crest graft
(three cases), and costal cartilage

transplantation to repair the condylar


region (one case).
According to a report by Johnson et al.,
aggressive growth characteristics of JOF
lesions are mainly observed in the maxilla.3 The anatomy of the maxilla prevents
the tumours at this site from being removed easily and completely, resulting
in a high rate of relapse and difficulties
with reconstruction. In this study, among
patients who had lesions in the maxilla,
only one (case 8) had single-side maxilla
resection and fibula flap transplantation.
The remaining eight patients who

Juvenile ossifying fibroma of the jaw

375

References

Fig. 7. Panoramic radiographs of juvenile ossifying fibroma (JOF): case 3. (A) Before surgery,
JOF presented as a single transparent shadow in the right mandible. There were spots of highdensity shapes. The boundaries were clear. The mandibular cortex had continuous edges. The
adjacent roots were displaced. (B) Two years after removal of the tumour, bone had formed at
the lesion site. There was no evidence of recurrence.

underwent reconstruction had mandibular


defects. At the end of follow-up, all
patients with maxillofacial reconstruction
had recovered their appearance and there
were no signs of recurrence. Therefore, it
is speculated that reconstruction can be
performed concurrently with preserving
the safety margins of the excision. Although there has been no report of malignant transformation, the physician should
be more cautious when choosing the timing of reconstruction for rapidly growing
lesions.17
In summary, JOF of the jaw is a type of
uncommon benign fibro-osseous lesion. In
the Chinese population, JOF is more commonly seen in females than in males and
may occur in children or adolescents. JOF
has two histological subtypes, which are
JPOF and JTOF; JTOF is more common in
the jaw. Its imaging characteristics include
a radiolucent or mixed density appearance
marked by clear boundaries. The diagnosis requires consideration of a combination of factors such as age, growth pattern,
imaging features, and pathology. Although JOF has aggressive characteristics
and a high recurrence rate (40%, 6/15),
malignancies have not been reported.
Therefore, initial conservative surgical
treatment is recommended, but a radical
approach is warranted for lesions that
behave destructively, that present rapid

growth, where there is pain and displacement of teeth, and for lesions with one or
more recurrence. In addition, as the recurrence rate following conservative treatment is high at up to 66.7% (6/9), longterm follow-up and vigilance is indicated.
Funding

This work was supported by research


grants from Shanghai Jiao Tong University Cross Research Fund for Medical and
Science (YG2012MS42), the Science and
Technology Commission of Shanghai Municipality (15411950300), and the Shanghai Natural Science Foundation of China
(13ZR1457100).
Competing interests

None.
Ethical approval

Due to the retrospective nature of this


study, it was granted an exemption in
writing by the Institutional Review Board
of the Ninth Peoples Hospital.
Patient consent

Not required.

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Sidransky D. World Health Organization
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Address:
Liqun Xu
Department of Oral and MaxillofacialHead
and Neck Oncology
Ninth Peoples Hospital
Shanghai Jiao Tong University School of
Medicine
Shanghai 200011
China
Tel: +86 21 23271699 5161;
Fax: +86 21 63166731
E-mail: maxilla@sina.com

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