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Case Report

Mandibular mass in pediatric patient:


A diagnostic dilemma
Abstract
A case of aggressive Juvenile ossifying fibroma in a child has
been discussed. The unique appearance of this particular lesion
demonstrates a close relationship between aggressive ossifying
fibroma and well-differentiated osteogenic sarcoma. The age of
the patient and the location of the tumor in the case presented
made it mandatory to utilize multiple diagnostic parameters
and adjunctive diagnostic help to resolve the dilemma posed
by this tumor. Differentiation of ossifying fibroma from other
benign and malignant neoplasms as well as fibrous dysplasia
is important in correct management of the lesion.

Ranadive PA, Deshpande MD, Ingole SN,


Ankush C1

Department of oral surgery, Nair Hospital Dental College,


Mumbai, 1Government Dental College, Mumbai, India
Correspondence:
Asst. Prof. P.A. Ranadive, 107, 1st floor, Nair Hospital Dental
College, A. L. Nair Road, Mumbai Central,
Mumbai- 400008., India.
E-mail: drpallavir@yahoo.com
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Website:
www.jisppd.com
DOI:
10.4103/0970-4388.100013

Aggressive Juvenile ossifying fibroma

PMID:
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Introduction
In 1872, Menzel first described the entity known as
ossifying fibroma,[1] although it can resemble focal
cement-osseous dysplasia radiographically and to a
certain extent histopathologically ossifying fibroma is
a true neoplasm with significant growth potential.[2] Its
affliction to the jaws can be related to the vast amount
of mesenchymal cellular induction that is required
in this region for odontogenesis; the possibility of
induction or genetic alteration leading to a neoplasm
is therefore greater.[3]
They expand slowly and asymptomatically, and
characteristically behave in a benign fashion. Occasionally
they may occur in a gigantic form exhibiting an
aggressive nature although none have been known to
metastasize.[4]
This tumor is mainly found in the tooth bearing
areas, with women in their twenties and thirties being
commonly affected; however, the younger and older men
are also affected.[3]

The term juvenile ossifying fibroma has been


introduced as a less common andmore aggressive
variant that occurs primarily in children and young
adults; such lesions have a rapid growth rate with
local aggressive tendencies, occasionallywith growth
mimicking sarcomas.[5] Histologically two patterns of
Juvenille ossifying fibroma are accepted (1) trabecular
(2) psammomatoid. Both varieties reveal similar
radiographic features and growth patterns, the trabecular
form is diagnosed initially in young patient. Both patterns
occur in either jaws but reveal a maxillary predominance.
Psammomatoid variant frequently appears outside the
jaws with over 70% arising in the orbit, frontal bones,
and para nasal sinuses.[2]

Case Report
A 5-year-old girl reported to the Department of
Oral and Maxillofacial Surgery, Nair Hospital Dental
College with a chief complaint of swelling in the
anterior region of the mandible. Patients mother
first noticed the swelling that was progressively

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Ranadive, et al.: Pediatric tumor

enlarging for 3 months. There was no history of


trauma to that area, no pain associated with the
mass, no tooth pain or mobility and she also denied
numbness to the lower lip. The patients medical and
dental histroy was non contributory. The swelling
was firm and nontender, palpable over the chin and
the submental region. Minor discomfort was elicited
on palpation; there were no palpable cervical lymph
nodes, bruits nor pulsations were demonstrated.
Intraorally a firm nontender swelling was seen
extending from 7282 in the buccal vestibule. There
was no lingual cortical expansion. Displacement of
teeth were seen [Figure 1].

Radiographic examination
OPG and CT scan showed an irregular radiopaque
lesion over the symphysis region with intermittent
radiolucent areas and ill defined peripheries.
There was no evidence of any root resorption,
but permanent dentition showed displacement
[Figures 2-4].

Subsequent course
An incisional biopsy was taken under General
anesthesia from the submental region [Figure 5].The
histologic diagnosis given was osteogenic sarcoma.
Because this did not correspond to the surgeons
clinical evaluation, the specimen was sent for a second
opinion to two different places. The diagnosis given
was desmoplastic fibroma and a rhabdomyosarcoma.
As two out of three histopathology reports were
suggestive of malignancy, incisional biopsy was
repeated at one of the referral centers. This specimen
was subjected to histochemical studies and was
found negative to Desmin,MyoD1, CD34, ruling out
embryonic rhabdomyosarcoma, spindle cells were
negative for S100, SMA, CD34. The final diagnosis
made based on the above-mentioned findings was
Juvenile ossifying fibroma. A locally aggressive
tumor necessitating excision.Based on clinical and
radiographic findings, correlated with the histologic
appearance, it was considered that the lesion was
an aggressive yet probably benign fibro-osseous
lesions. The mass was then excised in Toto. The
lingual cortex of the mandible appeared to have

Figure 1: Preoperative intraoral view

Figure 2: Preoperative orthopantomogram

Figure 3: Preoperative axial view CT scan

Figure 4: Preoperative coronal view CT scan

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Ranadive, et al.: Pediatric tumor

Figure 5: Intraoperative tumor exposed

Figure 7: Postoperative orthopantomogram

been eroded superficially due to the pressure of the


growing lesion. The patients postoperative course was
uncomplicated. Patient was kept under observation
for clinical and radiographic features of recurrence.
One year postoperatively, the patient has remained
asymptomatic [Figure 6].Root development is
normal. Radiographs of the patient showed complete
filling of the defect with normal bone and without any
evidence of recurrence [Figure 7].

Pathologic diagnosis
The final diagnosis was Juvenile ossifying fibroma.

Discussion
In evaluating the aggressive forms of ossifying
fibroma, it is important not only to carefully examine
the histologic appearance of the tumor but to strongly
consider the clinical radiographic and surgical
presentation in attempting to differentiate it from
a possible malignant entity. Several investigators
have reported the problems associated with the
differentiation between ossifying fibroma and
osteogenic sarcoma. [1,6,7] The patient described

Figure 6: Postoperative intraoral view

earlier provided the same diagnostic dilemma.


Occurrence of rapid growth, paresthesia, pain, and
tooth mobility are common features of osteogenic
sarcoma.[8,9] In our initial assessment of the patient,
the only clinical features that was of concern was
the apparent rapi d rate of growth. Although this
finding is not a typical characteristics of ossifying
fibroma, their presence in the more aggressive form
is acknowledged.[1,7,10] Radiographically the lesion
exhibited local aggressiveness, a diffuse scattering
and calcification. Although these characters are
representative of both the aggressive ossifying
fibroma and osteogenic sarcoma. The existence of
well-defined sclerotic borders and displacement of
the tooth structures rather than resorption supported
a diagnosis of a benign lesion.Appearance of the
tumor during surgery probably represented the
most significant support for a benign diagnosis. The
tumor mass was easily shelled out and erosion of the
cortical plates was seen. Perforation of cortical plates
that often is a feature of potentially malignant lesion
were not noted. These characteristics in combination
with clinical and radiographic findings influenced the
decision to consider this a benign lesion.The histologic
features exhibited by the lesion also generated a
distinct difference of opinion regarding whether it was
a benign or malignant . Most sections had microscopic
appearance that satisfactorily filled the basic criteria
for diagnosis of ossifying fibroma. The cellular soft
tissue component had no significant vascularity or
pleomorphism and was not particularly anaplastic in
appearance. In addition the mitotic index in sections
was low.Despite the unusual histologic presentation
the classification of this lesion as an aggressive
ossifying fibroma is appropriate and consistent with
previously reported cases. As is typical of many

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Ranadive, et al.: Pediatric tumor

fibroosseous lesions, proper differentiation between


the various pathologic entities is often an exercise in
judgment.

References
1. Walter JM Jr, Terry BC, Small EW, Matteson SR, Howell RM.
Aggressive Ossifying fibroma of Maxilla: Review of literature
and report of case. J Oral Surg 1979;37:276-86.
2. Drad W Neville, Douglas Damm, Carl Allen, Jerry Bouquot. Oral
and Maxillofacial pathology second edition saunders 2004.
3. Oral and Maxillofacial pathology a rationale for diagnosis and
treatment Robert E. Marx, Diane Stern. Quintessence publishing
Co,Inc.
4. Reaume CE, Schmid RW, Wesley RK. Aggressive ossifying
fibroma of mandible. J Oral Maxillofac Surg 1985;43:631-5.
5. Sciubba JJ, Younai F. Ossifying fibroma of mandible and maxilla:
Review of 18 cases. J oral Pathol Med 1989;18:315-21.

6. Hamner JE 3rd, Scofeild HH, Cornym J. Benign fibro-osseous


jaw lesions of periodontal membrane origin.An analysis of 249
cases. Cancer 1968;22:861-78.
7. Small IA, Goodman PA, Giant cemento ossifying fibroma of
maxilla: Report of case and discussion. J Oral Surg 1973;31:113-9.
8. Cocke WM Jr, Wade WM Jr. Osteogenic Sarcoma of the
Mandible. Oral Surg Oral Med Oral Pathol 1970;30:601-6.
9. Wilcox JW, Dukrat RC, Kolodny SC, Jacoby JK. Osteogenic
Sarcoma of mandible: Review of literature and report of a case
J. Oral Surg 1973;31:49-52.
10. Hamner JE 3rd, Lightbody PM, Ketcham AS, Swerdlow H.
Cemento-ossifying fibroma of the maxilla. Oral Surg Oral Med
Oral Pathol 1968;26:579-87.
How to cite this article: Ranadive PA, Deshpande MD, Ingole
SN, Ankush C. Mandibular mass in pediatric patient: A diagnostic
dilemma. J Indian Soc Pedod Prev Dent 2012;30:179-82.
Source of Support: Nil, Conflict of Interest: None declared.

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