Fibrous Dysplasia of The Jaw Bones - Sri Siddhartha Dental College

Fibrous Dysplasia of Jaw Bones

Volume 2 Issue 1 Febraury 2011

Case Report

Fibrous

Dysplasia

of

The

Jaw

Bones:

Clinical,

Radiographical and Histopathological Features. Report of Two Cases. Dr. Jyothi Mahadesh1, Dr. Charan Gowda2, Dr. Laxmi Devi3, Dr. Kokila G4. 1

Prof. and Head,

2

Reader,

3

Senior Lecturer,

4

Professor Department of Oral

Pathology, Sri Siddhartha Dental College, Tumkur, Karnataka.

Abstract: Fibrous dysplasia is a developmental benign bone lesion characterised by the replacement of normal bone by excessive proliferation of cellular fibrous connective tissue which is slowly replaced by bone, osteoid or cementum like material. It causes bone pain, deformities and pathological fractures. It may either be monostotic or polyostotic. Here we present two cases of fibrous dysplasia affecting mandible and maxilla( involving the sinus) respectively. Key words:- Fibrous Dysplasia, Monostotic, Polyostotic, Craniofacial Journal of Dental Sciences & Research 2:1: Pages 18-25

Introduction

its

onset

mainly

in

children

FD is a disturbance of bone

younger than 10 years of age.

metabolism that is classified as a

Signs and symptoms of FD include

benign fibro-osseous lesion. The

bone pain, pathological fractures

fibrous connective tissue containing

and bone deformatives. Laboratory

abnormal

findings

include

bone(1).

alkaline

phosphatase rarely, but

Fibrous displasia (FD) is a sporadic

calcium , parathyroid harmone , 25

benign skeletal disorder that can

hydroxy

affect one bone (monostotic form)

dihydroxyvitamin D levels in most

or

cases

bone

multiple

replaces

bones

normal

(polyostotic

vitamin

are

form). Gender prevalence of FD is

transformation

equal. The monostotic form is more

usually

common and affects 20-30 years of

therapy(2).

age while the polyostotic form has 18 Journal of Dental Sciences and Research

elevated

D,

normal. is

rare

serum

1,25-

Malignant and

is

precipitated by radiation


Etiology

is


a post zygomatic ,

After

decalcification

the

somatic mutation of GNAS gene

histopathology revealed. A highly

which encodes the α

cellular connective tissue stroma

Subunit

of

the

ubiquitious

resembling

fibroblasts

that

are

stimulatory G protein(3).

spindle shaped, plump and benign

Case report 1.

along with some osteoid like tissue

A 20 year old male patient reported

to

the

maxillofacial

department

surgery

of

at

Sri

with osteocytes but mostly not lined

by

shapes.

osteoblasts Along

in with

various this

Siddhartha Dental College with the

extravasated RBCs also seen(fig2).

chief complaint of swelling in the

A diagnosis of fibro-osseous lesion

lower

was made. Since cemento-osseous

back

teeth

region.

The a

fibroma and other fibro-osseous

diffuse bony hard swelling with no

lesions have similar histopatholgy,

tenderness in lower left 1/3rd of the

after correlating with the clinical

face

features

clinical

examination

measuring

2X3cm,

revealed

approximately

extending

antero-

posteriorly from angle of mandible

features

and

radiographical

a diagnosis of

Fibrous

Dysplasia was made.

region,

The lesion was surgically excised

superio-inferiorly from 2cm below

as there was erosion of lingual

ala tragal line to 0.5cm medially to

cortical plate and the specimen was

the lower border of mandible.

submitted

to

the

para-symphysis

CT scan revealed a mixed

examination.

for The

histopathological histopathology

radiolucent and radiopaque lesion

confirmed the diagnosis of fibrous

in left mandible from canine to

dysplasia. The case was negative

third

for both the syndromes.

molar

with

diffused

borders(fig1). An incisional biopsy

The follow-up is being done since

was made and the tissue was sent

one year and so far no recurrence

to the department of oral and

is seen. patient is healthy and fit

maxillofacial histopathological

pathology

for

examination.

19 Journal of Dental Sciences and Research


Volume 2 Issue 1 Febraury 2011 glass appearance in right maxillary sinus including the alveolar process and zygomatic process(fig3). The clinical

impression

was

Fibro-

osseous lesion. an incisional biopsy Fig

1:-

showing

mixed

radiolucent

and

was made from a representative area

radiopacities with lingual cortical Erosion.

and

was

histopathological the

sent

for

examination

department

of

oral

to and

maxillofacial pathology. After

decalcification,section

revealed Fig-2

showing

bony

trabaculae

and

hypercellular fibrous Connective tissue stroma.

a

connective

stroma

which was cellular, fibrous with plump cells. It was intermixed with bone of varying sizes and shapes.

Case report 2. A

24

year

Under low magnification Chinese old

male

patient

reported with the chief complaint of swelling in the right side of the face

since

examination asymmetry

2

years.

revealed on

right

Clinical a

facial

mid

face

region in the maxilla measuring 5X3 cm extending supero-inferiorly from right infra orbital margin till upper alveolar region and anteroposteriorly from right canine fossa to the right zygomatic arch. The swelling was bony, firm and well

letter

pattern

could

be

appreciated(fig4).

Features

were

suggestive of Fibro-Osseous lesion. After

clinical

radiological

features features

correlated

it

Fibrous

Dysplasia.

recontouring

was

was

and were

diagnosed

as

Surgical

done

for

the

patient. The

follow-up

is

being

done

regularly since 6 months and so far no recurrence is seen. Patient is healthy and fit.

defined.The skin over the swelling was normal. CT scan revealed an expansile bony lesion with ground 20 Journal of Dental Sciences and Research



Fig 4:- showing delicate bony trabeculae and

Fig 3:- showing ground glass appearance.

cellular connective tissue.

Table 1. Summary of cases of Fibrous Dysplasia in Sri Siddhartha Dental College And Hospital Tumkur. Case

Sex

Site

Radiographic appearance

Male

Age (years) 20

1

Left mandible

Mixed radiolucent/radio opaque appearance

2

Male

24

Right Maxillary zygomatic processes

sinus + alveolar

The

Ground glass appearance

activating

mutations

occur

Discussion:-

post zygomatically , replacing the

Fibrous dysplasia is defined as a

arginine residue amino acid with

benign

either a

osseous

characterised

by

a

disease process

of

cystein

amino

or

acid.

a

The

histidine mutation

normal bone resorption, followed

selectively inhibits GTPase activity,

by an abnormal proliferation of a

resulting in constitutive stimulation

disorganised fibro-osseous tissue.

of

It

intracellular

represents

about

7%

of

all

benign osseous tumors and may affect any bone

(4)

.

AMP-protein

Kinase

signal

A

transduction

pathways. This

mutated

protein

The etiology of F.D is a genetic

receptor

non-inherited condition caused by

function

mutation in the gene GNAS on

parathyroid hormone receptor, in

chromosome 20, that encodes the

skin

alpha subunit of the stimulatory G

stimulating hormone receptor, in

protein – coupled receptor, GSa.

ovaries


complex

coupled

in

through

through

autonomous

bone

through

melanocyte-

the

follicle-



stimulating hormone receptor and

and the swelling was asymptomatic.

in thyroid and the pituitary gland ,

Both the cases were in the third

through the thyroid and growth

decade of life and were males. In

hormone receptors respectively(2).

case

FD is classified depending on the

mandible while in case 2 the lesion

number of affected bones and the

involved

presence

craniofacial

or

skeletal

absence

of

extra-

abnormalities.

The

1

the

the

swelling

was

maxilla

complex.

in

and

the

The

age

group corresponded with the other

monostotic form affects only one

studies.

bone and corresponds to 70-80%

The radiographic appearance in F.D

of F.D cases. The polyostotic form

varies greatly depending upon the

corresponds to 20-30% of cases.

stage into 3 types,

The craniofacial type in which only

Type

the

craniofacial

involved

including

I-

a

complex

are

multilocular

the

and

well

jaw

small

unilocular

radiolucency

with

circumscribed

or a

border

maxilla. The craniofacial bones are

containing a network of fine bone

more affected in the polyostotic

trabeculae.

(50-100%)than in the monostotic

Type II- similar but with increased

form(20%)-

(4)

trabeculation

.

rendering

a

more

The other subtypes of polyostotic

opaque and mottled appearance.

F.D show café-au-lait pigmented

Type III- the lesion is quite opaque

skin lesion and endocraniopathies,

with

when

they

delicate

trabeculae

called

Jaffe-

giving a ground glass or Peau

syndrome

and

d’orange appearance to the lesion.

are

Lichtenstein

many

McCune-Albright syndrome.

This type is not well circumscribed

The presenting symptoms in both

but blends with the normal bone(5).

our

increasing

In our cases, case 1 showed mixed

swelling of the jaw bone that was

radiopaque /radiolucent lesion with

disfiguring

diffused border blending with the

cases

was

and

an

intraorally

the

swelling was bony hard and the

normal bone anteriorly.

overlying mucosa appeared normal 22 Journal of Dental Sciences and Research



Case 2 showed an expansile bony

Therefore for both the cases , the

lesion

clinical

with

ground

glass

features

appearance in the maxillary sinus

features

as

and

features were correlated to come

the

to

well

as

zygomatic

the

alveolar

processes

with

a

and

radiographic

final

decision

border merging with normal bone.

dysplasia.

Both

Conclusion:-

the

cases

had

type

III

histopathological

of

fibrous

radiographic presentation.

The fibrous dysplasia is significant

Based on the histopathology alone

for the dentists because it may

it

to

affect the facial, cranial and jaw

fibrous

bones leading to many deformities

is

virtually

differentiate

impossible

between

dysplasia and ossifying fibroma.

and

For this reason X-ray studies are

fibrous dysplasia are committed

indispensable for the interpretation

osteogenic

of fibro-osseous lesions of jaws. The

histopathology

(6)

shows

dysfunctions.

cells

The

with

cells

of

impaired

capacity to form normal bone. The mutated protein not only affects

considerable variation. The lesion

osteoblasts

may be a fibrous one made up of

various hormone receptors leading

proliferating

a

to endocrinopathies and café –au-

interlacing

lait spots. Both our cases did not

Irregular

show any skin pigmentations or

compact collagen

fibroblasts

stroma

of

fibres.

in

but

can

also

affect

trabeculae of bone are scattered

endocrinopathies.

through out the lesion with no

transformation occurs infrequently

definite pattern of arrangement.

with reported frequencies ranging

Some

may

from 0.4-4%. Both our cases have

show Chinese letter pattern. The

not shown any malignant changes

trabeculae may be coarse woven

till date and the follow up is still on

bone or lamellar bone which over

going.

time

of

will

these

trabaculae

mature

craniofacial type

especially

the

(5)

.


Malignant


Volume 2 Issue 1 Febraury 2011 Shafer’s text book of oral

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Corresponding Author:Dr. Jyothi Mahadesh, Professor and Head Department

Of

Oral

&

Maxillofacial Pathology Sri Siddhartha Dental College & Hospital Agalakote, Tumkur-572107 Email

id:

[email protected]
