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Maxillofacial FIBRO-

OSSEOUS LESIONS

Dr. Kranti Kiran Reddy E


Associate Professor
MNR Dental College and Hospital
Sangareddy
• The fibroblasts and collagen constitute the
"fibro-"
• Osteoblasts and bone constitute the
"-osseous" of "fibrosseous lesions"
Microscopic similarities and dissimilarities
among fibro-osseous lesions

Fibrous element variations

• Homogeneous plump Ossification (trabeculation) variations


monomorphic fibroblasts, • Metaplastic woven bone
hypercellularity, • ‘‘Chinese/Hebrew’’ figure trabeculae
• thin collagen fibers • Lamellar bone trabeculae
• Mature, hypocellular • Osteoblastic rimming
• Fasiculated, Storiform • Mosaic resting/reversal lines
• Trabecular paralleling
• Cemental woven
• Cemental microlamellar
• Sharpey fiber fringe
• Droplet (psammomatoid)
• Curvilinear conglomerates (‘‘Ginger
root’’)
Classification Schemes of Fibro-Osseous Lesions

 Charles Waldron Classification Of The Fibro-Osseous Lesions Of The Jaws


(1985)
 Working Classification Of Fibro-Osseous Lesions By Mico M. Malek (1987)
 Peiter J. Slootweg & Hellmuth Muller (1990)
 WHO Classification (1992)
 Waldron Modified Classification Of Fibro-Osseous Lesions Of Jaws (1993)
 Brannon & Fowler Classification (2001)
 WHO Classification Of Fibro-Osseous Lesions Of Jaws (2005)
 Paul M. Speight & Roman Carlos Classification (2006)
 Eversole Classification (2008)
Classification of fibro-osseous lesions of the
maxillofacial region.
• Fibrous dysplasia
• Monostotic fibrous dysplasia
• Polyostotic fibrous dysplasia
• Craniofacial fibrous dysplasia

• Osseous dysplasias
• Periapical osseous dysplasia
• Focal osseous dysplasia
• Florid osseous dysplasia
• Familial gigantiform cementoma
• Ossifying fibroma
• Conventional ossifying fibroma
• Juvenile trabecular ossifying fibroma
• Juvenile psammomatoid ossifying
fibroma

Speight P.M, Carlos R. Maxillofacial fibro-osseous lesions .Current Diagnostic Pathology


.2006;12:1-10
Fibrous Dysplasia
FIBROUS DYSPLASIA

Arrest of bone development in woven bone stage


that fail to mature into lamellar bone.

Normal medullary bone is replaced by abnormal


fibrous connective tissue
GENETICS AND PATHOGENESIS

• Postzygotic mutation of GNAS1(Guanine nucleotide binding


protein, α-stimulating activity polypeptide 1) gene (20q13.2)
Before 6th week of At 6th week of After 6th week
development development of development
CLASSIFICATION
ACCORDING TO NUMBER OF BONES INVOLVED

MONO OSTOTIC
POLY OSTOTIC
CRANIOFACIAL

ACCORDING TO RADIOGRAPHIC APPEARANCE (FRIES-1957)

PAGETOID TYPE (56%)


SCLEROTIC TYPE (23%)
CYSTIC TYPE (21%)
Pagetoid type
Sclerotic type
Cystic mass
ACCORDING TO TYPE OF TISSUES (Peter Ward Booth)

• SCLEROTIC
• FIBROOSSEOUS
• VASCULAR
• CYSTIC
• FIBROVASCULAR

A FURTHER SUBTYPE WHICH IS FAMILIAL AND PRESENTS IN


EARLY LIFE

• CHERUBISM
CLINICAL FEATURES
Monostotic- 80-85%

Site - jaws, ribs, long bones, pelvis

Age - 1-2nd decade

Sex - equal distribution

Maxilla > Mandible (Body)

Maxillary lesion may extend to-


zygoma, sphenoid, sinuses, orbit
(CRANIOFACIAL FIBROUS DYSPLASIA)
Polystotic-
15-20%
Age – 1-2nd decade
Site – Skull, facial bones, jaws, spines, shoulder girdle ,

pelvis

Associated syndromes-

Jaffe-Liechtenstein Syndrome - Polystotic FD


Cutaneous
pigmentation
2. McCune Albright Syndrome –
Polystotic FD
Café-au-lait spots
Endocrinal disturbances

3. Mazabraud Syndrome - Polystotic FD


Soft tissue myxoma
RADIOGRAPHIC APPEARANCE

• DIFFUSE MARGINS

• NO ROOT RESORPTION, DISPLACEMENT RARE

• FUSIFORM EXPANSION OF BONE

• GROUND GLASS
Roentgenographic Features
MRI
CT SCAN
Tc-99 Bone scan
HISTOLOGY

• CELLULAR FIBROBLASTIC STROMA

• IRREGULAR TRABECULAE OF WOVEN BONE


(CHINESE LETTER PATTERN)

• BONY TRABECULAE NOT SURROUNDED BY OSTEOBLASTS


Histopathology
Histopathology
Histopathology

Fibrous dysplasia, typical histology shows quite evenly distributed fine branching
trabeculae of woven bone.
Histopathology
A mature lesion of fibrous dysplasia. Lamellar bone is arranged in parallel arrays.
The stroma is typically moderately cellular with sparse collagen production
Histopathology
CHERUBISM
(FAMILIAL / CONGENITAL FIBROUS DYSPLASIA)

Autosomal dominant genetic defect

Gene responsible for cherubism – chromosome 4p16, SH3BP2


gene

Pathogenesis –

Affect remodeling of bone in the specific anatomic limit of


embryonic mandible and sometimes mandible and maxilla
FEATURES –

Manifestation 2 - 5yrs of age Fully expressed - 5yrs of age

Symmetrical involvement – clinically & radiographically

Chubby face

Heaven looking eyes

Nasal obstruction

Multiple missing / misplaced teeth


HISTOLOGY
OSSEOUS DYSPLASIA

ETIOPATHOGENESIS - ???????

Periodontal ligament
or
Disorders in metabolism of cells forming bone/cementum

Classified as —
Periapical osseous dysplasia
Focal osseous dysplasia
Florid osseous dysplasia
PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA

Synonyms - Cementoma, osseous dysplasia, cemental dysplasia

Associated teeth – Vital

Age – Above 30 yrs ( seldom below 20yrs)

Predilection for middle aged black women


RADIOGRAPHICALLY -

STAGE I - OSTEOLYTIC STAGE II – CEMENTOBLASTIC STAGE III - MATURE


HISTOLOGICALLY –

Mixture of fibrous,bone,cementum and cementum like tissues

TREATMENT - No treatment required


FOCAL OSSEOUS DYSPLASIA
• Site - Solitary lesions involving posterior mandibles

• Age – 4-5th decade

• Sex - females > males

• Reported more commonly in Whites

• Associated teeth - Vital


• RADIOGRAPHICALLY-

Well demarcated radiolucency


or
Mixed radiopaque and radiolucent regions
HISTOLOGY –
Fibrovascular stroma
Irregular trabeculae of woven bone and/or cementum

TREATMENT –
Not required
On surgical exploration – gritty, haemorrhagic
FLORID OSSEOUS DYSPLASIA (FLCOD)
(Sclerosing Osteitis / Diffuse Chronic Sclerosing Osteomyelitis /
Gigantiform Cementoma)

Alter bone pattern in bilateral fashion

Site – Tooth bearing area

Associated teeth - Vital

Specially affect black adult females

Asymptomatic jaw expansion may be noted


Diffuse mixed radio-opacity and radiolucency
HISTOLOGY –

Admixture of woven bone trabeculae and droplets of


cementum in fibroblastic stroma
Familial gigantiform cementoma

• It is a disorder of gnathic bone that ultimately leads to the


formation of massive sclerotic masses of disorganized
mineralized material.

• Autosomal dominant - variability in phenotypic expression


• Caucasian families
• Identical to those seen in FLCOD
• Manifests at an early age – first decade
Familial gigantiform cementoma
Ossifying fibroma

• Previously termed as cemento-ossifying fibroma.


• This was coined on the basis that most lesions may be
associated with the teeth or may contain cementum-like
spherical calcifications.

WHO classification
• Conventional ossifying fibroma
• Juvenile trabecular ossifying fibroma
• Juvenile psammomatoid ossifying fibroma
Ossifying fibroma

• occurs in the tooth-bearing areas of the jaws

• odontogenic in origin - arises from tissues of the


periodontal membrane.

• slight predilection for the posterior mandible


CLINICAL FEATURES –
Age – 2-4th decade

Sex – Females > males

Site – Tooth bearing areas of jaw


(Body of mandible)

Solitary lesions

Causes gradual expansion of cortical plates – Bony hard swelling


It presents as a well-circumscribed radiolucency with varying degrees of
calcification, but is rarely opaque.
Radiographically, the lesions are expansile and
radiolucent with internal floccular to dense opacities
RADIOGRAPHICALLY

Sharp border between lesion and normal bone

Early lesions Maturing lesion Mature lesions


HISTOLOGY
Woven bone with osteoblast rim
Pattern of mineralization varies from place to place
Variable vascularity
Sometimes encapsulated
Histopathological features

• well-demarcated lesion
that is separated from
the overlying cortical
bone by a thin zone of
fibrous tissue.
• Biopsies that include
the margin of the lesion
are therefore invaluable
to enable diagnosis
• A key feature of ossifying fibroma distinguishes it from fibrous dysplasia
JUVENILE AGGRESSIVE OSSIFYING FIBROMA

A variant of ossifying fibroma (cemento-ossifying fibroma)

More aggressive
Different anatomic site
• JTOF
• JPOF
CLINICAL FEATURES –

Age – Below 15 years (85%)


Site – Maxilla, orbit, Frontal, Ethmoid
Rapid expansion of cortical plate

Facial asymmetry
Proptosis
Exopthalmos
Visual disturbances
Nasal obstruction
RADIOGRAPHICALLY

Well demarcated
Primarily radiolucent lesion
Histopathological features

• The trabecular variant is


composed of densely cellular
fibrous tissue with little collagen,
containing thin strand-like
trabeculae of osteoid and woven
bone.
• Osteoblasts are prominent and
may be incorporated into the
osteoid material.
• psammomatoid variant is
also densely cellular but
the calcifications are
spherical or lamellated
like typical psammoma
bodies

• They may have a


‘feathered’ or ‘fluffy’
margin where the osteoid
merges or projects into
the cellular stroma—
sometimes in a thorn-like
pattern
Differences between fibrous dysplasia and
ossifying fibroma.
parameters that distinguish among the benign fibro-osseous lesions
Disease Clinical features Radiologic findings Histopathology

Fibrous dysplasia Expansion of bone Diffuse radiolucent or Trabecular


Unilateral, painless ground glass ‘‘Chinese/Hebrew’’
Alk phosphatase
Mono or Polyostotic
Osteitis deformans Expansile lesions Ground glass Rare sarcoma &
Alk phosphatase Cotton wool giant cell lesions
Cranial neuropathies Mosaic bone
Polyostotic
Hyperparathyroidi Parathormone, Ca Ground glass Trabecular
sm Renal disease Multilocular Brown Giant cell lesions
‘‘Big head disease’’ tumors
Massive opaque jaw
lesions
Focal cemento- Non-expansile Focal circumscribed apical Trabecular
osseous Painless Lucent or target lesions Cementifying
Dysplasia
Florid cement- Non-expansile Multiquadrant opacities Trabecular
osseous African descent Associated bone cavities Sclerotic
Dysplasia
Disease Clinical features Radiologic findings Histopathology

Ossifying fibroma Expansile, painless Circumscribed lucent or target Trabecular


lesion
Not otherwise specified Rarely multifocal Root divergence Cementifying
Jaws

Ossifying fibroma Expansile, painless Circumscribed lucent Trabecular


Trabecular variant Root divergence Floccular opacities Giant cell foci
Aggressive Fibroplasia
Ossifying fibroma Expansile , Facial bones Circumscribed Psammoma
Psammomatoid Aggressive Dense floccular opacities
Massive, expansile
Gigantiform cementoma Multiquadrant Well delineated lucent with
Often familial floccular opacities Trabecular
Cementifying

Focal sclerosing Non-expansile Apical well delineated Trabecular


Osteomyelitis Painless Lucent, target or opaque Sclerotic
Carious tooth
Diffuse sclerosing Expansile Diffuse ground glass Trabecular
Osteomyelitis Painful Proliferative periostitis Sclerotic
Dental source of
infection + or –
THANK YOU

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