Juvenile aggressive ossifying fibroma /prosthodontic course

INTRODUCTION
(Active juvenile ossifying fibroma, juvenile aggressive
cemento-ossifying fibroma )
The juvenile ossifying fibroma is a unique lesion that
has been distinguished from the larger group of
ossifying fibroma on the basis of age of patient ,most
common site of involvement and clinical behaviour.
It is grouped under “fibrous –osseous neoplasms” in
modified classification of benign fibro-osseous lesions
(waldrone 1993)
Histologically , the lesion is non-encapsulated but well
demarcated from surrounding bone .Two distinct
histopathologic variants are-
1.Psammomatoid juvenile ossifying fibroma(Psjof)
www.indiandentalacademy.com

Psammomatoid juvenile ossifying fibroma
PsJOF- is a benign bone tumor of the
craniofacial skeleton occuring predominantly
but not exclusively in children &was first
reported by Benjamins in 1938
Termed Psammomatoid ossifying fibroma of
the nose and paranasal sinuses by Gogl in
1949.

• JUVENILE AGGRESSIVE-
OSSIFYING FIBROMA—A
CASE REPORT

12yr. old girl visited the department of
oral medicine & radiology, Guru Nanak
Dev Dental College Sunam ,with the
chief complaint of swelling on right
side of face since 3 years.

History of presenting illness
Swelling first appeared at the age of 6 years
as a small pear shaped one which was painless
in nature & gradually increased in size with in
the span of 2 years .
Patient underwent surgery for that & no records
of surgery were available with patient .
Patient remained asymptomatic for one year and
again similar painless swelling recurred at same
site and gradually increased within the span of
3 years to attain the present size.
 H/o Slight difficulty in breathing and
swallowing .
 H/o watering from right eye, no visual and
auditory disturbances.
 No H/o of trauma, pus discharge &
paresthesia.

Past medical and Family history were
noncontributory.
Personal history
patient was vegetarian ,brushed her teeth twice
daily following horizontal strokes with no
deleterious oral habits.

General examination
Patient was well oriented ,moderately built
& nourished with no pallor, icterus &
clubbing with normal height and weight.
No enlargements of salivary glands and
lymphnodes were palpated .
 All vital signs were within normal
limit.

EXTRAORAL EXAMINATION
 Gross Facial asymmetry was
appreciated on the right middle
1/3rd
of face due to solitary ,well
–defined swelling measuring 8X 9
cm in dimension & roughly oval
in shape.
 Superiorly , starts from infra
orbital ridge and extends
inferiorly about 2cm above the
lower border of mandible.
Medially starts from left ala of
nose crosses the mid sagittal line
and extends 3cm in front of right
tragus .
Overlying skin was stretched with
focal areas of hyperpigmentation.www.indiandentalacademy.com

Deviation of nose towards
left side .
 Right Eye raised, drooping of
right angle of mouth.
Obliteration of inferior orbital
and nasolabial fold.
On palpation all the
inspectory findings were
confirmed.
Swelling was hard in
consistency with
compressibility & egg shell
crackling present at superior
and medial margins .

Intraoral Examination
A solitary well-defined swelling
obliterating the right buccal vestibule
involving most of the hard palate
except on left lateral margin
Starting anteriorly 2 mm away from
palatal gingival margin of maxillary
anterior teeth and extending
posteriorly upto the junction
of hard and soft palate.
 laterally from right buccal
vestibule, crosses the alveolar ridge
and midpalatal raphae extending 1.5
cm in front of palatal gingival margin
of left maxillary posterior teeth.www.indiandentalacademy.com

On Palpation Swelling was hard in
consistency with Egg-shell crackling in the
center of swelling.
Hard tissue examination
Revealed patient was in Mixed
dentition stage
11,21, were drifted and showing
grade I mobility.
16 -Missing

VITALITY TEST- all teeth in the vicinity of lesion
were vital.
All haematological and serological investigations were
within normal limit except Elevated alkaline
phosphatase level. -(244 IU/l)

HAEMATOLOGICAL EXAMINATION
Hb - 12gm%
TLC 9800 /cumm
DLC - Neutrophil -71%,
Lymphocyte-23%
Monocyte-2%
Eosinophil -04%
Basophil –nil
ESR - 05 mm (1st
hour)

RADIOGRAPHIC
INVESTIGATIONS

MAXILLARY OCCLUSAL
CROSS-SECTIONAL VIEW
OCCLUSAL RADIOGRAPH
of MAXILLA
Dense homogeneous well
demarcated radiopacity seen
on right side with
expansion of buccal cortex
and palatal bone.
Extending from right
premaxilla to beyond the
right maxillary tuberosity .
Buccally placed 12,13,14

• OPG reveals – on right side homogeneous radiopacity
involving maxilla encroached maxillary sinus ,zygomatic
bone , nasal cavity, pterygoid plates.
• Downward slanting of Occlusal plane with Drifting of
maxillary teeth of right side.
• Multiple retained
52,53,54,55,62,63,64,65,73,74,75,83,84,85 teeth..
• Missing 16,17
• Erupting teeth 23,24,25 27,33,34,37,43,44 47www.indiandentalacademy.com

PNS
PNS VIEW
Reveals dense
homogeneous
radiopacity involving
right maxilla,
zygomatic buttress,
maxillary
sinus,ethmoidal
sinus .Obliteration of
right nasal fossa.
Medially it crosses
midline
Superiorly extending
about 0.5 cm above
right infra orbital rim
Inferiorly approaching
the alveolar process of

CT SCAN

• Right maxillary sinus is
grossly expanded with
distorted contures and
marked thinning of all
walls.
• Expansile mixed
attenuation mass with
well demarcated outline
filling the right maxillary
sinus, frontal sinus,
ethmoidal sinus ,right
nasal cavity.
Coronal
section www.indiandentalacademy.com

Coronal section
• Medially crosses midline
• Superiorly extending
toward inferior orbital
ridge.
• Inferiorly encroaching the
palate on right side.
• Left maxillary sinus is
well pneumatised.

AXIAL SECTION
Swelling
Extending
toward
sphenoid sinus

Radiographic Differential
diagnosis
• Fibrous dysplasia
• Cemento-ossifying fibroma
• Squamous cell carcinoma
• Osteogenic sarcoma
• Calcifying epithelial odontogenic tumour

HISTOPATHOLOGICAL EXAMINATION
• Incisional BIOPSY
was taken and
histopathological
section showed
proliferating
fibroblastic stroma.
• with psammoma like
masses resembling
cementum.

BASED ON HISTORY,CLINICAL
EXAMINATION,RADIOGRAPHIC AND
HISTOLOGIC EXAMINATION final
diagnosis OF
JUVENILE AGGRESSIVE OSSIFYING
FIBROMA WAS MADEwww.indiandentalacademy.com

Management
• Surgical currettage was done.

DISCUSSION

• The fibro-osseous lesions are those in which the
normal bone architecture is replaced by fibroblasts
and collagen fibers that contain various amounts
of mineralized material, and these lesions include
a broad group of several entities like OF, JOF,
FD and so on.
• Most of them have been considered as benign
lesions, but JOF has been classified as a different
disease because of its local aggressive behavior
and its tendency to predominantly occur in
children and adolescents.

• Review of the literature shows that it is
difficult to establish definitive diagnosis
criteria for JOF.
The main characteristics are:
patient under 15 years of age
the location of the tumour
the radiologic pattern
tendency to recur.

JOF is often seen in a very young child. In
reviews published by HAMNER et al. and
SLOOTWEG et al., the mean age of onset was
11.5 and 11.8 years , respectively, correspond to
our case.
Clinically, Most cases of maxillary JOF are
asymptomatic.
The first clinical manifestation is a swelling of
the maxilla. When the orbital bone and paranasal
sinuses are involved, the patients may develop
exophthalmos, bulbar displacement and nasal
obstruction. In large lesion , there may be root
resorption, displacement of teeth in the tooth-

• The sites of JOF in our case was consistent with
other reports, in that JPOF occurred in the
paranasal sinus, calvarium, maxilla and mandible.
• The radiologic features are variable and depend on
the tumour’s location and the amount of calcified
tissue produced by the tumour, and the lesion will
show varying degrees of radiolucency.

• JOF can be easily excluded from malignant
bone tumors on the routine histological
examination. According to the WHO
classification, the cementum-like ossicles in
JPOF have a thick irregular collagenous
rim. An extremely cellular fibrous stroma
tended to be observed in JOF, which may be
attributed to the aggressiveness of JOF
according to other reports.

Conclusion
Juvenile aggressive ossifying fibroma of
maxilla is an uncommon and rare
lesion.Cosmetic and occlusal problems are
often first manifestations, because of its
confounding clinical ,radigraphic and
histopathologic pattern ,diagnosis of JOF
is challenging. Proper diagnosis and
timely intervention is essential for better
prognosis.

REFERENCES
• Robert B. Brannon and †Craig B. Fowler ,Benign Fibro-Osseous Lesions: A Review
of Current Concepts . Advances in Anatomic Pathology Vol. 8, No. 3, pp. 126–143.
• Samir El-Mofty Psammomatoid and trabecular juvenile ossifying
fibroma of the craniofacial skeleton: Two distinct clinicopathologic
entities, Oral Surg Oral Med Oral Pathol Oral Radiol Endod
2002;93:296-304
• Ah Hup M. Ong, Chong Huat Siar, Cemento-ossifying fibroma with
mandibular fracture. Case report in a young patient Australian Dental
Journal 1998;43:(4):229-33
• Nabil J. Khoury et al Juvenile ossifying fibroma: CT and MR findings .Eur Radiol
(2002) 12:S109–S113
• H. K. Williams et al Juvenile ossifying fibroma. An analysis of
eight cases and a comparison with other fibro-osseous lesions ;J Oral Pathol Med
2000: 29: 13–8
• Sohyung ParkJuvenile Ossifying Fibroma: A Clinicopathologic Study of 8 Cases
and Comparison with Craniofacial Fibro-osseous LesionsThe Korean Journal of
Pathology 2007; 41: 373-9

Juvenile aggressive ossifying fibroma /prosthodontic course

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