10.Fibrosseous lesions of the jaw.pptx

Fibrosseous lesions of
the jaw

INTRODUCTION
Characterized by replacement of
normal bone by fibrous tissue
containing a newly formed
mineralized product.
Can be
Hamartomatous,
dysplastic processes,
and neoplasms
Group of lesions are known to
encompass common characteristics that
include common clinical, radiographic
and microscopic features.
Confusing area
in diagnostic
pathology

Charles Waldron Classification Of The Fibro-
Osseous Lesions Of The Jaws (1985)
1. Fibrous Dysplasia
a. Monostotic
b. Polyostotic
2. Fibro-Osseous (Cemental) Lesions Presumably Arising In The Periodontal Ligament
a. Periapical Cemental Dysplasia
b. Localized Fibro-Osseous-Cemental Lesions (Probably Reactive In Nature)
c. Florid Cement-Osseous Dysplasia (Gigantiform Cementoma)
d. Ossifying & Cemenifying Fibroma
3. Fibro-Osseous Neoplasms Of Uncertain Or Detectable Relationship To Those Arising In The
Periodontal Ligament
a. Cemetoblastoma, Osteoblastoma & Osteoid Osteoma
b. Juvenile Active Ossifying Fibroma & Other So Called Aggressive, Active Ossifying /Cementifying
Fibromas.

Classification Schemes of Fibro-Osseous
Lesions
1. Charles WaldronClassificationOf The Fibro-Osseous Lesions Of The Jaws
(1985)
2. Working ClassificationOf Fibro-Osseous Lesions By MicoM. Malek (1987)
3. Peiter J. Slootweg & Hellmuth Muller (1990)
4. WHO Classification(1992)
5. WaldronModifiedClassificationOf Fibro-Osseous Lesions Of Jaws (1993)
6. Brannon& Fowler Classification(2001)
7. WHO ClassificationOf Fibro-Osseous LesionsOf Jaws (2005)
8. Paul M. Speight & RomanCarlos Classification(2006)
9. Eversole Classification(2008)

Peiter J. Slootweg & Hellmuth Muller (1990)
In 1990 Peiter. J. Slootweg & Hellmuth Muller gave a classification that laid emphasis primarily
on the histopathological features, and they underscore that this classification requires inclusion
of adjacentnormal bone to make diagnosis. However in the absence of this, the clinical &
radiological features have to be taken in to consideration.
Group I: Fibrous Dysplasia
Group II: Juvenile Ossifying Fibroma
Group III: Ossifying Fibroma
Group IV: Periapical Cemental Dysplasia & Florid Osseous
Dysplasia

Waldron Modified Classification Of Fibro-
Osseous Lesions Of Jaws (1993)
Later on, to overcome the demerits of his own classification, Waldron
reviewed the subject of benign fibro-osseous lesions of jaws (BFOL) in
1993 and suggested a modification of his earlier classification.

Brannon & Fowler Classification (2001)
•In 2001, Brannon & Fowler gave
another classification which was
quite different from that of
Waldron & WHO classification.
This was done to include more
number of lesions which were also
showing features like FOL.

WHO Classification Of Fibro-Osseous Lesions
Of Jaws (2005)
1) Ossifying Fibroma (OF)
2) Fiberous Dysplasia
3) Osseous Dysplasia
a. Periapical Osseous Dysplasia
b. Focal Osseous Dysplasia
c. Florid Osseous Dysplasia
d. Familial Gigantiform Cementoma
4) Central Giant Cell Granuloma
5) Cherubism
6) Aneurismal Bone Cyst
7) Solitary Bone Cyst

Paul M. Speight & Roman Carlos Classification
(2006)
This new classification
Concentrated on the
HISTOPATHOLOGICAL
FEATURES that may guide the
working surgical
Pathologist towards a diagnosis.

Definitive diagnosis can rarely be
rendered on the basis of
histopathological features alone
rather procurement of a final
diagnosis is usually dependent
upon assessment of microscopic,
clinical and imaging.

Introduction
Normal medullary bone
and cortices are
replaced by a
disorganized fibrous
woven bone
Resultant bone is more
elastic and weaker than
the original bone
Caused due to mutation
in GNAS gene
No evidence to suggest
a hereditary influence
Defect in
osteoblastic
differentiation
and maturation

Etiology
Mutation in the GNAS1
gene
G protein activated
Over production of IL6
Over-production of
cAMP
Prevents
differentiation of cells
within the osteoblastic
lineage
Surrounding
osteoclasts activated
Expansion

If the mutation occurs
during early embryologic
life
Multiple bone lesions,
cutaneous pigmentation
and endocrine
disturbances
(MCCUNE ALBRIGHT
SYNDROME)
If the mutation occurs during
later stages
Multiple bone lesions
(POLYOSTOTIC)
If the mutation occurs
during postnatal life
Affects single bone
(MONOSTOTIC)

Monostotic fibrous dysplasia
• 75% of fibrous dysplasia cases.
• rib (28%), femur (23%), tibia,
craniofacial bones (10–25%), and
humerus.
• Seen most frequently in the body of
the mandible or in the premolar-molar
regions of the maxilla.
• Degree of bone deformity is relatively
less severe compared with that
of the polyostotic type.

CLINICAL FEATURES
• Gender predilection : Males = females
• More common in children and young adults
• First clinical sign : painless swelling or bulging of the jaw.
• Tipping or displacement of the teeth due to the progressive expansile
nature of the lesion
• Slow growth, become static with skeletal growth

FIBROUS DYSPLASIA OF THE
MAXILLA
• Serious form of the disease
• Not well circumscribed
• Extend locally to involve the maxillary sinus,
the zygomatic process and the floor of the
orbit, and even extend back toward the base
of the skull.
• Severe malocclusion and bulging of the canine
fossa or extreme prominence of the zygomatic
process, producing a marked facial deformity,
are typical sequelae of this disease.

RADIOGRAPHIC FEATURES
Three basic patterns
• FIRST : small unilocular radiolucency or larger multilocular
radiolucency, both with a rather wellcircumscribed border and
containing a network of fine bony trabeculae.
• SECOND : more opaque and typically mottled in appearance.
• THIRD: opaque with many delicate trabeculae gives a ‘groundglass’ or
‘peau d’orange’ appearance to the lesion.

THE CORTICAL BONE
BECOMES THINNED
BECAUSE OF THE
EXPANSILE NATURE OF
THE GROWTH

POLYOSTOTIC FIBROUS DYSPLASIA
• More frequently involves the skull and facial bones, pelvis, spine, and
shoulder girdle.
• Although the polyostotic variety tends to occur in a unilateral
distribution, involvement is asymmetric and generalized when disease
is bilateral.
• Often, the initial symptom is pain in the involved limb associated with
a limp, spontaneous fracture, or both.

• The structural integrity of the
bone is weakened, and the
weight-bearing bones become
bowed.
• The curvature of the femoral
neck and proximal shaft of the
femur markedly increase
causing a Shepherd’s crook
deformity, which is a
characteristic sign.

TYPES OF POLYOSTOTIC FIBROUS
DYSPLASIA
Polystotic
fibrous
dysplasia
‘Café-au-
lait’ spots
Jaffe’s
Syndrome
Cutaneous pigmentation in polyostotic fibrous
dysplasia is ipsilateral to the side of bony
lesions, a feature that differentiates this
disease from pigmentation in
neurofibromatosis.

Polystotic
fibrous
dysplasia
‘Café-au-
lait’ spots
Endocrino
pathies
McCune
Albright
Syndrome
Polystotic
Fibrous
dysplasia
Intramus
cular
myxoma
Mazabraud’
s syndrome
The relationship between fibrous dysplasia and myxoma remains unclear, whereas an underlying
localized error in tissue metabolism has been proposed to explain this occasional coexistence.

CRANIOFACIAL FORM
• 25% of patients with the monostotic form and in 50% with the
polyostotic
form. It also occurs in an isolated craniofacial form.
• Sites of involvement most commonly include the frontal, sphenoid,
maxillary, and ethmoidal bones.
• Hypertelorism, cranial asymmetry, facial deformity, visual
impairment, exophthalmos, and blindness may occur because of
involvement of orbital and periorbital bones.
• Involvement of the sphenoid wing and temporal bones may result in
vestibular dysfunction, tinnitus, and hearing loss.
• When the cribriform plate is involved, hyposmia or anosmia may
result.

RADIOGRAPHIC PRESENTATION
• Nearly all cases of fibrous dysplasia will show a diffuse, hazy
trabecular pattern that has been called the ground‐glass appearance
pattern as radiolucent
• CT scan pictures of fibrous dysplasia show a homogeneous, finely
trabecular bone pattern replacing the medullary bone and both
cortices and often the lamina dura as well.
It shows greater buccal than lingual expansion and does not displace
the inferior alveolar canal.

• Usual appearance of fibrous dysplasia
in long and short tubular bones
includes a lucent lesion in the diaphysis
or metaphysis
• The lucent lesion has a thick sclerotic
border and is called the rind sign.

HISTOLOGIC FEATURES
• Considerable microscopic variation
• Fibrous one made up of proliferating fibroblasts in a compact stroma
of interlacing collagen fibers
• Irregular trabeculae of bone are scattered throughout the lesion with
no definite pattern of arrangement.
• Characteristically, some of these trabeculae are C-shaped, or as
described by one author, chinese character-shaped.

DIFFERENTIAL DIAGNOSIS
• Most important differential diagnosis for fibrous dysplasia is to
distinguish it from an ossifying fibroma.
• Other entities that may resemble fibrous dysplasia include
1. Chronic sclerosing osteomyelitis,
2. Paget disease, and
3. Sometimes osteosarcoma.

TREATMENT
• Timing of intervention is based on the symptoms manifesting as a result of the
disease.
• Recommended treatment options can be divided into 4
categories:
1. Observation
2. Medical therapy
3. Surgical remodelling/paring procedure/sculpting
procedure
4. Radical excision and reconstruction
Radiation therapy is contraindicated, as it carries the risk of post irradiation
bone sarcoma.

OBSERVATION
• Monitoring is through serial radiographs, CT scans, and clinical
examinations.
• The preferred approach monostotic fibrous dysplasia and craniofacial
fibrous dysplasia is no treatment.
• If osseous contouring surgery is desired, it is ideal to defer it until
adulthood (ages 18 to 21 years)
• Decreased nerve function may be an indication for surgical therapy.

MEDICAL TREATMENT
Currently, no medical therapy exists for the permanent
cure of fibrous dysplasia.
1. Bisphosphonates.
2. Systemic steroids

• Pamidronate given intravenously in 500 mL of normal saline at a dosage
of 30 mg over 4 hours for 3 consecutive days. Response to this therapy
lasts for 3 to 6 months, then the dosage must be repeated.
• The major effect of BISPHOSPHONATE THERAPY was decreased bone
pain and improved skeletal bone strength, drug has even slowed down
bony expansion in some patients.

• Rare, inherited, developmental abnormality
that causes bilateral enlargement of the jaws,
giving the child a cherubic facial appearance.
• Usually develops at 2-6 years of age
characterized by painless bilateral swelling of
the posterior mandible.
• Cherubic look- child assumes a cherubic facial
appearance with upward displacement of
globe and exposure of scleral rims (eye to
heaven appearance).
• First described by Jones in 1933.

Classification: Arnott 1978
• Grade I – Bilaterally
involvement of mandibular
ascending rami.
• Grade II - Involvement of both
maxillary tuberosities as well
as mandibular ascending rami.
• Grade III – Involvement of the
whole maxilla and mandible
except coronoid and condylar
processes.

Clinical features
•Swelling firm and hard on palpation and
overlying mucosa intact and non painful.
• Numerous dental abnormalities: agenesis
of second and third molars of mandible,
displacement of teeth, premature
exfoliation, transposition and rotation of
teeth.
• In severe cases, tooth root resorption
occurs.
• Maxillary ridge involved: V shaped palate.

Radiographic features
• Bilateral multilocular cystic expansion of the jaws.
• Margins are well defined, well corticated and smooth around most of the
radiolucency.
• Floating tooth syndrome: presence of numerous unerupted teeth and
destruction of alveolar bone may displace the teeth.

Histopathologic features
• Collagenous stroma, which contains a large number of spindle shaped
fibroblasts.
• Lesion usually reveals numerous multinucleated giant cells.
• In long standing cases (resolving lesions) the tissue becomes more
fibrous, the number of Giant cells decreases & new bone formation is
seen.

TREATMENT
• The treatment of cherubism should be based on the known natural
course of the disease and the clinical behavior of the individual case.
• Treatment of cherubism is usually conservative and into teenage
years is devoted to trying to aid in eruption of teeth.
• Later it is directed towards cosmetic recontouring of the affected
bones, which is carried out in 3rd decade.

• Also called as Osteitis deformans.
• Characterized by excessive and abnormal remodeling of bone.
• Named after Sir James Paget in 1876 who described the clinical course
of the disease.
• Etiology: Still unknown.
• Possible etiology suggested are: Autosomal dominant inheritance, viral
infection, inflammatory cause, autoimmune connective tissue disorder.

CLINICAL FEATURES
• Prevalence increases with age, most common after 50 years
of age.
• M = F
• Clinical features are variable and depends on which bones are
affected.
• Commonly affects pelvis and spine and may also involve femur,
fibula, tibia, bones of hand and ribs.

• Most common presenting complaint is pain which is perceived as a
dull aching pain below the soft tissues.
• Other signs and symptoms are pathologic fracture, nonspecific
headache, impaired hearing, platybasia, paresthesia, gait difficulties,
enlargement of skull.
• Involvement of facial bones is occasionally seen which gives lion like
appearance called as Leontiasis ossea.

• Maxilla: exhibits progressive enlargement, alveolar ridge becomes widened
and palate is flattened if teeth are present they may become loose and
migrate.
• Mandible: findings are similar but less aggressive than maxilla.
• Extraction sites heal slowly and risk of excessive bleeding from
extraction site.

RADIOGRAPHIC FEATURES
• Paget’s diseases has three radiographic stages:
An early radiolucent resorptive stage.
A granular or ground glass appearing second stage.
A denser, more radiopaque appositional late stage creating a cotton
wool appearance.

• The overall density of the jaws may decrease or increase
depending upon the number of trabeculae.
• The isolated lesion in the skull referred to as osteoporosis
circumscripta.
• Dental changes: hypercementosis of roots, loss of lamina
dura, obliteration of PDL space and resorption of roots

Histopathologic features
• Depends on the stage of the disease (osteoclastic or osteoblastic)
• In the active resorptive stages, numerous Osteoclasts surround
the bone trabeculae
• Simultaneously, osteoblastic activity is seen with formation of
osteoid rims around bone trabeculae
• Characteristic picture of Paget’s disease is presence of
hematoxyphilic reversal lines. These lines indicate the junction
between resorptive & formative phases of the bone and results in
a “jigsaw puzzle” or “mosaic bone” appearance

Treatment
• Medical treatment consists of use of Calcitonin or bisphosphonates to
inhibit bone resorption.
Calcitonin can be taken either by nasal spray or subcutaneously and
bisphosphonates are taken orally or by intravenously.
• Surgical management is directed towards cosmetic and/or functional
recontouring of bones.
• Causes of death in patients with paget’s disease are left heart failure and
osteosarcomas.
• Sarcomatous changes has been reported in 5-15% patients with
paget’s disease.

OSSIFYING FIBROMA
(CEMENTO- OSSIFYING FIBROMA)

• A well-demarcated, encapsulated, expansile intraosseous lesion of the
jaws composed of cellular fibrous tissue containing interconnecting
bony trabeculae lined by osteblasts with spherical calcifications and
irregular, randomly oriented bony structures.
• Etiology is unknown
• 3 types:
Cementifying – More of cementum
Ossifying - More of bone
Cemento-ossifying – Admixture of both

Clinical features
• Slow growing benign neoplastic lesion
• More often in jaws especially mandible in
90% of cases
• Site: Molar > Premolar >Incisors >Canine
region
• More common in women(5:1) in 20s or
40s but can be found in younger & older
men.
• Asymptomatic
• Can be ulcerated by occlusion when
achieves larger size

• Well defined unilocular radiolucency, mixed radiolucent
radiopaque,and radiopaque lesions.
• Cortical expansion without perforation

Treatment and prognosis
• Circumscribed nature of the ossifying fibroma generally
permits enucleation with ease.
• It shells out from the surrounding bone.
• Grown large lesions can be surgically resected and bone
grafting done.
• Very good prognosis, and no evidence of malignant
transformation till date.

• Trabecular form
– Diagnosed initially in younger patients.
– The mean age of trabecular juvenile ossifying fibromas is
approximately 11 years
• Psammomatoid – At 22 yrs of age.
– Appears outside the jaws, with over 70% arising in the supraorbital,
ethmoid and frontal bones and paranasal sinuses.

Clinical Features
In early to late childhood
• Maxilla more often than mandible
• In Maxilla : Canine fossa & zygomatic arch
• Singular, rapid-growing, painless swelling
• Overgrowth of tissue that occurs centrally in the jaws
• May involve impacted or unerupted teeth
• Increased level of serum alkaline phosphatase
• Severe maloccusion
• Nasal obstruction, epistaxis, exophthalmous and rarely intracranial
extension.

• Unilocular or multilocular radiolucency with well defined borders.
• Radiolucent or mixed radiolucent and radiopaque appearance
(ground glass)
• Lamina dura is usually obscured and the cortical plates thinned

• Management and prognosis are uncertain.
• Smaller lesions - complete local excision or thorough curettage
appears adequate.
• Rapidly growing lesions - wider resection may be required and
adjuvant Interferon alpha therapy for 1 yr.
• Recurrence rates - 30% to 58%

CEMENTO OSSEOUS DYSPLASIA
• Cemento-osseous dysplasia occurs in the tooth bearing areas of the
jaws and is probably the most common fibro-osseous lesion
encountered in clinical practice
• Histopathologic features share many similarities with fibrous
dysplasia and ossifying fibroma
• Arises in close approximation to the PDL and exhibits histopathologic
similarities with the PDL, and some investigators have suggested these
lesions are of PDL origin.

CEMENTOBLASTOMA
Clinical Features
1. usually 2nd or third decade,
2. usually before age 25
3. mandible in first premolar to molar region
4. pain, swelling, and expansion of the cortical plates
5. continuous with root, which resorbed

Radiographic Features
1. radiopaque mass confluent with the roots of the associated tooth
2. surrounded by radiolucent line
3. loss of normal PDL area and root outline
4. root resorption of the apical third

• Slight posterior mandibular predilection
• Slight male predominance
• Painful or tender
• Size: 2-12cm
• Well circumscribed round solitary lesion expansile
• Mixed radiolucent radiopaque lesion not surrounded by sclerotic bone

Treatment
• Local excision or curettage
• A large surgical enbloc removal for large lesions
• Prognosis : Good
• May recur
• Transformation in to osteosarcoma

CENTRAL GAINT CELL GRANULOMA
• First described by Jaffe (1953)
• Benign lesion accounting for approximately 7% of all benign
tumours of the jaws (Tosco et al., 2009).
• The site most frequently involved is the
anterior part of mandible
• Females under 30 years of age.
• True biologic nature of the lesion is not
known.

Clinical features
• Aymptomatic slow growing swelling to
a tender aggressive lesion that cause
local bone lysis, root resorption or
tooth displacement
• CGCGs are classified into aggressive
and nonaggressive lesions based on
their biological behaviour, assessed by
the presence of pain, rapid growth,
perforation of the cortex, and a
tendency to recurrence

Histological features
• CGCG shows an intraosseous lesion consisting of fibro-cellular
tissue.
• It contains multiple foci of haemorrhage, aggregations of
multinucleated giant cells, and occasional trabeculae of
woven bone.
• Radiologically
– Multilocular or less frequently unilocular Radiolucency
– Margins : Well Demarcated / Scalloped border
– Expansion and thining of cortices is noted

Treatment
• The conventional treatment curettage.
• Non-surgical methods
– Systemic administration of calcitonin
– intra-lesional corticosteroids (introduced by
Jacoway et al.)
– administration of a-interferon
• En-bloc resection is the treatment which
provides the lowest recurrence rate and has
been suggested as the treatment of choice for
locally aggressive CGCGs.

Aneurysmal bone cyst
• Jaffe and Lichtenstein were the first to recognize ABC as an
intraosseous, osteolytic lesion, chiefly affecting the metaphyseal
region of long bones and vertebrae.
• Bernier and Bhaskar described the first case of ABC in the jaws in
1958
• ABC is a benign cystic lesion of bone, composed of bloodfilled
spaces separated by connective tissue septa containing fibroblasts,
osteoclast type giant cells and reactive woven bone

Clinical features
• It accounts for 1.5% of the nonodontogenic, nonepithelial
cysts of the mandible.
• Mandible > Maxilla (3:1)
• Body > Ramus > Angle.
• Age >20yrs M=F
• The ABC of the jaw is a psuedocyst lacking epithelial lining.

The radiological features of ABC
• Bone is expanded
• Appears cystic resembling a
honeycomb or soap bubble and is
eccentrically ballooned.
• There may be destruction or perforation
of the cortex

Histologically
• ABC consists of many sinusoidal blood filled
spaces set in a fibrous stroma, with
multinucleated giant cells and osteoid.
• Hemosiderin is present in variable amounts
and there is evidence of osteoid and bone
formation.

Treatment
• The treatment modalities are curettage, block resection and
reconstruction, radiotherapy and systemic calcitonin therapy.
• Self healing cases have also been reported
• Simple curettage recurrence rates varying from 21 to 50%.

Osteochondroma
• Osteochondroma or osteocartilaginous exostosis is characterized by a
cartilage capped osseous projection protruding from the surface of
the affected bone.
• It occurs in adolescence or young childhood, with 80% of cases being
in first 2 decades of life.
• M:F =1:1

Clinical features
• Osteochondromas arising from the jaw may
present as an asympotomatic bony hard
swelling
• If present in the condylar region may produce
– Unilateral open bite
– Chin deviation
– Facial asymmetry
• R/F
– Sessile /pedunculated lesion
– Continuous with the adjacent cortex and
medullary spaces
– Well defined radio-opacity

• Conservative approach / Surgical Excision
• Recurrence rarely reported

10.Fibrosseous lesions of the jaw.pptx

10.Fibrosseous lesions of the jaw.pptx

Recommended

Recommended

More Related Content

Similar to 10.Fibrosseous lesions of the jaw.pptx

Similar to 10.Fibrosseous lesions of the jaw.pptx (20)

Recently uploaded

Recently uploaded (20)

10.Fibrosseous lesions of the jaw.pptx

Editor's Notes