Fibro-osseous lesions of jaw

Fibro-Osseous Lesions Of The Jaws
Presented by: Guided by:
Dr. Sapna K Vadera Dr. S.R.Shenoi
(P.G. Student) (Prof, Guide and H.O.D)
Dept Of Oral And Maxillofacial Surgery ,VSPM’S Dental College, Nagpur

• Introduction
• Definition
• Fibrous dysplasia
• Cherubism
• Paget’s Disease
• Ossifying Fibroma
• Juvenile Ossifying Fibroma
• Cemento-ossifying fibroma
• Cementoblastoma, Osteoblastoma, Osteoid osteoma
• CGCG
• Anerysmal Bone Cyst
• Osteochondroma
• Hyperparathyroidsm
• Conclusion
CONTENTS

The term refers to a diverse process in which the normal architecture
of bone is replaced by fibrous tissue containing varying amount of
foci of mineralization. These group of lesions are known to
encompass common characteristics that include common clinical,
radiographic and microscopic features.
INTRODUCTION
Bahl S, Sandhu S , Gupta M. Benign Fibro‐osseous Lesions Of Jaws‐ A Review. International
Dental Journal Of Student’s Research. 2012;1(2):56-68

Most of these lesions are of unknown aetiology, while some lesions
are believed to be neoplastic while others are related to metabolic
disturbances and are also believed to cause considerable diagnostic
challenges.
While some of the FOL are believed to be true neoplasms having
a considerable potential for growth if not fully excised , the
peculiar anatomic location of these craniofacial lesions can result
in conditions like encephalitis and sometime meningitis which
are considered fatal.
INTRODUCTION

Histologically , these group of FOL are characterised by the
replacement of normal bone by fibrous connective tissue matrix. The
fibrous tissue displays variable features like varying degree of
mineralization in the form of woven bone or cementum like
basophilic structures which are indistinguishable from cementicles
INTRODUCTION

• Waldron described fibro osseous lesions as a group of
pathological changes with in the jaw bones in which normal
bone is replaced by fibrous tissue ,with or with out
calcification .
• Goaz & White Fibro osseous lesions are a group of
conditions that replace normal bone with benign fibrous tissue
containing variable amount of mineralization.
DEFINITION
Waldron CA. Fibro-osseous lesions of the jaws. J Oral Maxillofac Surg. 1985 Apr;43(4):249-62.

Benign mesenchymal skeletal tumors in which mineralized
tissue, blood vessels and giant cells in varying proportions
replace normal bone.
DEFINITION
Papadaki ME, Troulis MJ, Kaban LB. Advances in diagnosis and management of fibro-osseous lesions.
Oral and maxillofacial surgery clinics of North America. 2005 Nov 30;17(4):415-34.

• Charles Waldron Classification Of The Fibro-Osseous Lesions Of The Jaws
(1985)
• 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 SYSTEMS
Eversole R, Su L, ElMofty S. Benign fibro-osseous lesions of the craniofacial complex. A review. Head Neck
Pathol. 2008 Sep;2(3):177-202

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 (Category II)
a. Cemetoblastoma, Osteoblastoma & Osteoid Osteoma
b. Juvenile Active Ossifying Fibroma & Other So Called Aggressive, Active Ossifying
/Cementifying Fibromas.
Charles Waldron Classification Of The Fibro-
osseous Lesions Of The Jaws (1985)

Classification system proposed by Brannon and
Fowler(2001)
I. Fibrous dysplasia
A. Monostotic
B. Craniofacial
C. Polyostotic
D. McCune-Albright syndrome
II. Ossifying fibroma and juvenile ossifying fibroma
III. Osseous dysplasia
A. Periapical
B. Focal
C. Florid
D. Familial gigantiform cementoma
Eversole R, Su L, ElMofty S. Benign fibro-osseous lesions of the craniofacial complex. A review.
Head Neck Pathol. 2008 Sep;2(3):177-202

WHO Classification Of Fibro-Osseous
Lesions Of Jaws (2005)
1) Ossifying Fibroma (OF)
2) Fibrous 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
Eversole R, Su L, ElMofty S. Benign fibro-osseous lesions of the craniofacial complex. A review. Head
Neck Pathol. 2008 Sep;2(3):177-202

(RECENT) Eversole Classification, 2008
I. Bone dysplasias
a. Fibrous dysplasia
i. Monostotic
ii. Polyostotic
iii. Polyostotic with endocrinopathy (McCune-
Albright)
b. Osteitis deformans
c. Pagetoid heritable bone dysplasias of childhood
d. Segmental odontomaxillary dysplasia
II. Cemento-osseous dysplasias
a. Focal cemento-osseous dysplasia
b. Florid cemento-osseous dysplasia
Eversole R, Su L, ElMofty S. Benign fibro-osseous lesions of the craniofacial complex. A review. Head
Neck Pathol. 2008 Sep;2(3):177-202

III. Inflammatory/reactive processes
a. Focal sclerosing osteomyelitis
b. Diffuse sclerosing osteomyelitis
c. Proliferative periostitis
IV. Metabolic Disease: hyperparathyroidism
V. Neoplastic lesions (Ossifying fibromas)
a. Ossifying fibroma NOS
b. Hyperparathyroidism jaw lesion syndrome
c. Juvenile ossifying fibroma
i. Trabecular type
ii. Psammomatoid type
d.Gigantiform cementomas
(RECENT) Eversole Classification, 2008
Eversole R, Su L, ElMofty S. Benign fibro-osseous lesions of the craniofacial complex. A review.
Head Neck Pathol. 2008 Sep;2(3):177-202

FIBROUS DYSPLASIA
• Term coined by Louis Lichenstein in 1938
• First described by Von Recklinghausen in 1891
Ricalde P, Magliocca KR, Lee JS. Craniofacial fibrous dysplasia. Oral and maxillofacial surgery clinics of
North America. 2012 Aug 31;24(3):427-41.

• Also known as:
i. Fibrocystic disease
ii. Osteitis fibrosa localista
iii. Focal osteitis fibrosa
iv. Fibro-osteodystrophy
FIBROUS DYSPLASIA

• Fibrous dysplasia (FD) is a nonheritable, genetic disorder
characterized by the replacement of normal bone by immature,
haphazardly distributed bony and fibrous tissues.
• The resultant fibro-osseous bone is more elastic and structurally
weaker than the original bone.
• The HALLMARK of the disease is a solitary focal, or generalized
multifocal inability of bone forming tissue to produce mature
lamellar bone, and an arrest at the level of woven bone
FIBROUS DYSPLASIA

• The cause of this disorder is a gene mutation that prevents the
differentiation of cells within the osteoblastic lineage.
• Slow –growing lesion
• Median age of onset: 9.5 years
• Cause painless swelling of
affected bone
FIBROUS DYSPLASIA

Classification Of Fibrous Dysplasia By Kaban And
Colleagues
ON BASIS OF CLINICAL BEHAVIOUR AND RADIOGRAPHIC FINDINGS
1. Quiescent Leisons
2. Non-aggressive leisons
3. Aggressive leisons
QUIESCENT LEISONS :
 Seen in older patients
 Demonstrate no progressive growth
 Seen as radio-opaque leison on radiograph
NON-AGGRESSIVE LEISONS :
 Seen in teenagers around pubertal growth
 Demonstrate very slow growth
 Seen as mixed radiolucent radio-opaque leisons on radiograph
AGGRESSIVE LEISONS
 Appear in patients < 7 years age
 Large rapidly growing tumors accompanied with tooth displacement, root resorption, cortical
thinning and perforation
 May cause paresthesia by impinging nerves

Classification Of Fibrous Dysplasia
• First classification
Monoostotic
Polyostotic
a) Jaffe’s type
b) Albright type
• Second classification( acc to STEWART)
Monoostotic
Polyostotic
Albright syndrome
Monomelic ( Involvement of bone of one extremity)
Ricalde P, Magliocca KR, Lee JS. Craniofacial fibrous dysplasia. Oral and maxillofacial surgery
clinics of North America. 2012 Aug 31;24(3):427-41.

• G protein consist of 3 subunits G alpha, G beta and G gamma located to inner surface
of cell membrane.
• Alpha subunit is also bound to
GDP(inactive G protein) or
GTP( active G protein).
Pathogenesis of fibrous dysplasia
(molecular basis)

• G protein family includes five types on basis of 5 different alpha subunits which
are as follows :
Gs ( stimulatory of adenyl cyclase)
Gi (inhibitory of adenyl cyclase)
Gq (activates phospholipase)
Gt (tranducins 1 and 2)
21

Cascade of event that results in transmission of signal :
Binding of ligand to G-protein coupled receptor
Dissociation of alpha subunit from Gbeta Ggamma
and replacement of linked to Galpha GDP by GTP
Activation of adenyl cyclase by binding to active alpha
subunit
Formation of cAMPfrom ATP. cAMPis secondary
messanger that activates intracellular proteins.

• Eventually alpha subunit unwind from adenyl cyclase and link again to GβGγ,
which is caused by hydrolysis of GTP to GDP i.e G protein is rendered
inactive and ready to act again and repeat
cycle.
Schematic representation of the effects of Gsa mutation on adenylate cyclase
activity

So, overproduction of cAMP has 3 effects:
1. Hyperfunction of endocrine organs
2. Increased proliferation of melanocytes
3. Effect on differentiation of osteoblasts thus
leading to fibrous dysplasia
Pathogenesis of fibrous dysplasia

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 type of mutation takes place after birth, while the polyostotic type takes place during
the late stage of embryo development.

Clinical Features
• Following 4 patterns of disease are recognized :
1. Monostotic form (70-80%)
2. Polyostotic form (20-30%)
3. Craniofacial form
4. Cherubism

Monostotic fibrous dysplasia
• Single bone involved so less serious than polyostotic form and maily
affects jaws
• Occur in children and younger age group <10 years
• Equal gender distribution
• Site involved (in decreasing order) as described by SCHLUMBERGER
1. Ribs(28%)
2. Femur(23%)
3. Tibia
4. Craniofacial form (10-25%)
5. Humerus
Maxilla more commonly involved mainly posterio
region(Leontiasis ossea).
Most common area involved is premolar-molar region.
 Leisons of maxilla extend locally to involve maxillary sinus,
zygomatic process, floor of orbit, skull base producing marked
skull base deformity : CRANIOFACIAL FORM

Radiographic findings in monostotic
fibrous dysplasia
• 3 basic patterns may be seen:
 In one type, the lesion is generally a small unilocular radiolucency or somewhat larger
multilocular radiolucency both with well circumscibed border containing network of fine
trabeculae.

fibrous dysplasia
 In second type, the pattern is similar except that increased trabeculation renders the
lesion more opaque and typically mottled in appearance.

fibrous dysplasia
 Third type is a quite opaque type with many delicate trabeculae giving a ground glass or
peau d' orange appearance to the lesion. It is not well circumscribed and blends into
the adjacent normal bone.
NOTE : In all 3 types, generally cortical bone becomes thinned but seldom this bony plate is
perforated.
In CRANIOFACIAL FORM, there is characteristic roentgenographic thickening of the base of
the skull.

Histology
 Proliferation fibroblasts in compact
stroma of interlacing collagen fibres
 Irregular trabeculae scattered
throughout leison. Some trabecuale
are typically C,S shaped, JIG-SAW
PUZZLE or has chinese letter
appearance
 The trabeculae consist of immature,
nonlamellar (woven) bone without
osteoid rims or osteoblasts.

Polyostotic fibrous dysplasia
(Mccune albright syndrome)
• Involvement of two or more bones.
• M: F 1:3
• Bony sites affected in decreasing order of
frequency : Femur, skull, tibia, humerus, ribs,
fibula, radius, ulna, mandible,vertebrae.
• Polyostotic fibrous dysplasia + cafἐ au lait
pigmentation : Jaffe- Lichtenstein
syndrome.
• Polyostotic fibrous dysplasia + cafἐ au lait
pigmentation( irregular but regular in
neurofibromatosis) + multiple
endocrinopathies : McCune Albright
Syndrome.

irregular “coast-of-Maine” café-au-lait
spots on the thorax of a fourteen-year-old
girl with McCune-Albright syndrome
• Cutaneous pigmentation in PFD
is ipsilateral to the side of bony
lesions, a feature that
differentiates pigmentation of
this disease from that in
neurofibromatosis. The location
and shape of the spots usually
can help to distinguish between
them. The spots in McCune
Albright Syndrome have jagged
borders whereas those in
neurofibromatosis are
smooth,regular
Polyostotic fibrous dysplasia
(Mccune albright syndrome)

• Frequently identified deformities include
1. Coxa vara (Coxa vara is a deformity of the hip, whereby the angle between the head
and the shaft of the femur is reduced to less than 120 degrees. Normal 120-130)
2. bowing of the tibia
3. the Harrison groove (a horizontal depression along the lower border of the thorax,
corresponding to the costal insertion of the diaphragm)

Craniofacial fibrous dysplasia
• Occurs in 10-25% of patients with monostotic form and in 50% with the polyostotic
form.
• It also occurs in an isolated craniofacial form. In the isolated variety, no
extracranial lesions are present.
• It typically presents at around 10 years of age and then progress throughout
adolescence.

Craniofacial fibrous dysplasia
• Site of involvement most commonly include the frontal, sphenoid, maxillary and
ethmoidal bones. The occipital and temporal bones are less commonly affected.
• The clinical presentation depends on the site, duration, extent and nature of the
lesion.
• It ranges from a mild local swelling with little or no pain to a gross deformity.

Macroscopic Features/ gross patholgy of Fibrous
Dysplasia
• Surgical exposure of fibrous dysplasia reveals a yellowish
white tissue with a distinctive gritty feel, imparted by the
small trabeculae of bone scattered throughout the lesion.
• The lesion can be easily peeled away from the encircling shell
of reactive bone by blunt dissection, and lesions rarely, if ever,
penetrate the reactive shell and extend into soft tissue.

Diagnosis of fibrous dysplaisa
 Imaging studies including conventional radiography, CT scan
and MRI
 Nuclear scitigraphy
 Biopsy
 Molecular or mutational analysis of GNAS1 gene
 Lab studies to document endocrine disturbances

Radiographic Features
 Depends upon the stage of the
disease.
 Early onset lesions are radiolucent
and later progressively calcify,
culminating in a “Ground Glass” or
Mottled Mixed radiolucent/
radiopaque pattern.
 Critical feature to diagnosis- FD do
not have discrete margins; rather,
the lesion blends into the
surrounding normal appearing
bone.
EARLY
ADVNCED

Ground glass pattern
Cotton wool
Orange peel

CT SCAN

MRI

SCINTIGRAPHY
 At the initial presentation,
radionuclide bone scintigraphy is
useful to demonstrate the extent
of the disease.
 Actively forming lesions in
adolescents have greatly
increased isotope uptake.
 The isotope scan shows increased
uptake throughout life, but the
uptake becomes less intense as
the lesions mature.
A 16-year-old boy with McCuneAlbright
syndrome and involvement of virtually all
skeletal sites (panostotic) is shown.

Investigation of the gnas gene in
the diagnosis of fibrous dysplasia
 Done by direct sequencing of the GNAS gene.
 High prevalence of GNAS gene mutations in fibrous dysplasia.
 Through genetic amplification techniques such as polymerase chain reaction, it is now
possible to test for the genetic mutation in peripheral blood samples.
 Absence of GNAS mutation does not exclude fibrous dysplasia as the sample may not
contain mutant cells.
 This novel technique may have application in the diagnostic and therapeutic
monitoring of patients with fibrous dysplasia.

Differential Diagnosis
• Hyperparathyroidism may produce a similar pattern.
However, these diseases are polyostotic; bilateral; and, unlike
fibrous dysplasia, do not cause bone expansion.
• Paget ’s disease may produce a similar pattern and may cause
expansion, but it occurs in an older age group, and when it
involves the mandible, the whole mandible is involved, unlike
the unilateral tendency of fibrous dysplasia.

Cemeto-ossifyng fibroma trabeculae line by osteoblasts
Osteomyelitis may result in enlargement of the jaws, but the
additional bone is generated by the periosteum; therefore the new
bone is laid down on the surface of the outer cortex, and close
examination may reveal evidence of the original cortex within the
expanded portion of the jaw. Fibrous dysplasia, in contrast, expands
the internal aspect of bone, displacing and thinning the outer cortex
so that the remaining cortex maintains its position at the outer
surface of the bone. The identification of sequestra aids in the
identification of osteomyelitis.
Differential Diagnosis

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.

1. 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) Like cherubism, fibrous dysplasia
shows less growth and its activity is reduced as adulthood
approaches. Regrowth is most commonly seen when surgeries are
performed on patients younger than 21 years
• Special attention to cranial nerve function during monitoring of these
lesions should be exercised
• Decreased nerve function may be an indication for surgical therapy.

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

Why bisphosphonates in fibrous dysplasia which is a osteoblastic
lineage disorder???
• GNAS mutations result in abnormal proliferation and differentiation of bone
marrow stromal cells.
• In those osteoblastic cells, IL-6 secretion is increased as a result of GNAS
activation, with consequent activation of surrounding osteoclasts, allowing the
FD lesion to expand and create osteolytic lesions.
• A direct link has been established between the GNAS mutation in stromal cells
and IL-6 production, so that FD, which is an osteoblastic lineage disorder, is
also often associated with a hyperosteoclastic component. ( AS INTERLUKIN-6)
IS REQUIRED FOR NORMAL FUNCTIONING OF OSTECLASTS

 Although there is no evidence of abnormal osteoclastic activity,
bisphosphonate therapy has been utilized for patients with symptomatic
polyostotic disease
 Pamidronate given intravenously in 500 mL of normal saline at a dosage of 30
mg over 4 hours for 3 consecutive days.( total cumulative dose 90mg).
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.
Why bisphosphonates in fibrous dysplasia which is a osteoblastic
lineage disorder???

3. Surgical remodelling
• Chen and Noordhoff,and later Ricalde and
Horswell, emphasized an approach to the
treatment of craniomaxillofacial FD based
upon classification of the skull into 4 zones
and the relative reconstruction importance of
each zone
 ZONE 1 represents the fronto-orbito-malar
regions of the face. These are esthetically
critical and can be adequately reconstructed
with simple bone grafting techniques after
resection. For this region, they recommended
radical excision and reconstruction.
Chen YR, Noordhoff MS: Treatment of craniomaxillofacial fibrous
dysplasia: How early and how extensive? Plast Reconstr Surg 86:835, 1990

 ZONE 2 refers to the hair bearing scalp. It
is not typically an aesthetic concern, and
as such, intervention is optional for the
patient. Treatment :
Conservative or resection

• ZONE 3 refers to the central skull
base including the sphenoid,
pterygoid, petrous temporal bone,
and mastoid.
• Given the difficulty in obtaining
surgical access to these areas, the
authors recommended
observation of lesions in this
region.

• ZONE 4 comprises the tooth
bearing portions of the skull, the
maxilla and mandible.
• The authors recommend
conservative management, shaving
and recontouring. This is because
resection of teeth-bearing bones
will result in the patient having to
require dentures, which are less
functional than the original
dentition

• The supraorbital osteotomy is used
in the resection of lesions of the
superior half of the orbit.
• The neurosurgeon performs a
frontal craniotomy and removes the
bony plate to expose the frontal
lobe.
• With gentle retraction of the
frontal lobe and the placement of a
malleable plate to protect the
orbital contents, osteotomies are
made through the supraorbital rim
and orbital roof to remove the
segment of bone containing the
lesion.
• The osteotomies are made using a
combination of burrs and saws.
57

• The orbitomaxillary osteotomy is used
to resect lesions affecting
• lateral orbital wall
• maxilla
• zygomatic body
• zygomatic arch.
• A frontotemporal craniotomy combined
with osteotomies through the
superolateral orbit and zygomatic arch,
are made to facilitate the removal of
the lesion.
58

• For lesions involving the inferior
aspect of zone 1, the coronal flap
alone may be insufficient for
adequate exposure.
• In this case, a combination of
coronal flap and Weber–Ferguson
incision or degloving face incision
along the buccal sulcus is employed
• Osteotomies are performed
through the glabella, orbit, nasal
bones and maxilla above the level
of the dental roots.
• After the bone segment containing
the lesion is removed, the defect is
assessed for reconstruction.

• For defects involving the orbit, the goals of reconstruction are to
restore symmetric skeletal form, symmetric orbital position and
volume, normal anatomic compartmentalization, and to protect
vital structures (i.e., globe and brain).
• Split calvarial grafts can be used for reconstruction. Alternatively,
a segment of titanium mesh can be plated to the construct to
reconstruct one wall of the orbit.
• Edges of the bone graft are burred to ensure a flush fit with no
areas of prominence.
• Dural tenting sutures are placed to minimize dead space
between the dura and the graft

Dural tenting sutures. Sutures are placed through the dura and
tied; they are then passed through the holes drilled through the
bone graft and retied

• To avoid palpable instrumentation or even a visible prominence, use low-
profile plates and screws in an area anterior to the hairline.
• In placing bone grafts, a step is burred along the native bone margin, into
which the bone graft is inset.
• This is to provide additional support and increase the contact area
between the graft and the adjacent native bone.
inner cortex ledge medially (arrows) that allows inset of the
construct, and avoidance of a step-type defect.

ALGORITHM FOR MANAGEMENT OF COSMETIC DEFORMITIES FOR
ORBITOMAXILLARY FIBROUS DYSPLASIA.

Algorithm for management of functional deformities
of the orbital cavity

Algorithm for management of functional deformities
of the the nose and sinuses

Algorithm for management of functional deformities of the
dental alveolus

• Also called as familial fibrous dysplasia of jaws,
disseminated juvenile fibrous dysplasia, familial
multilocular cystic disease of jaws, familial fibrous swelling
of jaws.
• An autosomal dominant fibro osseous lesion of jaws that
stabilizes after growth period usually leaving some facial
deformity and malocclusion.
• First described by Jones in 1933.
CHERUBISM
Kau CH, Souccar NM, English JD, Kamel SG, Wong ME. The surgical and orthodontic management of
cherubism in a growing child. Journal of Cranio-Maxillofacial Surgery. 2012 Apr 30;40(3):229-33.

 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:
• Early childhood at the age of 2 - 4 years.
• M : F – 2 : 1
CHERUBISM

Lesion grading
Type-I
Type-II
Type-III

• Cherubic look- child assumes a cherubic facial appearance
with upward displacement of globe and exposure of scleral
rims (eye to heaven appearance).
• 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.
CHERUBISM

• Floating tooth syndrome: presence of numerous unerupted
teeth and destruction of alveolar bone may displace the
teeth.
• With adulthood the cystic areas in the jaws become re-
ossified results in patchy sclerosis and gives a ground glass
appearance.

 Histopathologic features:
• Collagenous stroma, which contains a large number of spindle shaped
fibroblasts.
• Numerous small vessels are present and the capillaries exhibits large
endothelial cells and perivascular cuffing.
• 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.
Miloro M, Ghali GE, Larsen P, Waite P. Peterson's principles of oral and maxillofacial surgery;
3rd edition; volume 1; PMPH-USA; 2004.

• 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.
Rajendran R. Shafer's textbook of oral pathology. Elsevier India; 2009.
PAGET’S DISEASE

 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.
PAGET’S DISEASE

• 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.
• Simian appearance: appearance and gait like apes.

• Waddling gait: short step gait.
• Maxilla: exhibits progressive enlargement, alveolar ridge
becomes widened and palate is flattened if teeth are present
they may become loose and migrates.
• Mandible: findings are similar but less aggressive than maxilla.
• Extraction sites heal slowly and risk of excessive bleeding from
extraction site.
• Osteogenic sarcoma may develop in patients with paget’s
disease- poor prognosis
PAGET’S DISEASE

 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 trabeculae are altered in number and shape, most
often they increase in number but initially they decreases.
White SC, Pharoah MJ. Oral radiology: principles and interpretation. Elsevier Health Sciences;
2014 May 1.
PAGET’S DISEASE

• 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
White SC, Pharoah MJ. Oral radiology: principles and interpretation. Elsevier Health Sciences;
2014 May 1.

 Laboratory findings:
• Elevated serum alkaline phosphatase levels.
Normal value in adult : 1.5 – 5.0 (Bodansky units)
In Paget’s disease : 50 – 250 (Bodansky units)
• Elevated urinary hydroxyproline levels- reflects increased
osteoclastic activity and bone resorption.
• Urinary N-telopeptide (NTX) and alpha-C telopeptide (CTX)
have emerged recently as sensitive biochemical markers of
bone resorption.- Increased

 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
PAGET’S DISEASE

• The marrow is replaced by fibrous connective tissue.
• Few cases show multiple small acellular bony masses that
fuse as the disease progresses.

 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

• 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
OSSIFYING FIBROMA
(CEMENTO- OSSIFYING FIBROMA)

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

Treatment & 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.

JUVENILE OSSIFYING FIBROMA
• 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)
Predominantly soft tissue consistency with varying internal
calcifications.
 Lamina dura is usually obscured and the cortical plates thinned

Treatment and Prognosis
• 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%
• Malignant transformation has not been documented.

• 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, so correct diagnosis can be problematic
• Etiology: mesenchymal stem cells
• 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.
CEMENTO OSSEOUS DYSPLASIA

Clinical Type Periapical COD Focal COD Florid COD
Clinical Features • In periapical region
of mand. anterior
teeth with non-
inflammed pulps.
• Size always <1cm
• Asymptomatic
• More common
• Size less than 1.5
cm
• Associated with
vital teeth or
edentulous areas
• Asymptomatic
• Extreme variant
• Occurs in bilateral
fashion
• Asymptomatic
• Cortical expansion
can be there
• Non-neoplastic,
self limited growth
Site • Mand. anterior
teeth
• In post. mandible • Mand.>Maxilla
• May involve all 4
quadrants
Sexual Predilection • Middle aged black
women
• 4th – 5th decade,
White women
• Adult black women
Associated
pathologies
• Idiopathic bone
cavity
• Idiopathic bone
cavity
• Osteomyelitis
D.D • Pulpal periapical
pathologies
• OF(Easily comes
out in big pieces)
• Osteomyelitis
• Paget’s Dis.
Treatment • No , only follow up • Biopsy and follow
up
• Reqd. if OM
develops.

Focal Cemento-osseous Dysplasia
Periapical Cemental Dysplasia

Florid Cemento-osseous Dysplasia
With Simple Bone Cysts
With Osteomyelitis

CEMENTOBLASTOMA, OSTEOBLASTOMA AND
OSTEOID OSTEOMA
• Osteoblastoma and Osteoid osteoma are recognized
neoplasms in the extragnathic skeleton
• Can be differentiated only on their clinical and radiologic
characteristics
• Cementoblastoma:
A benign, well-circumscribed neoplasm of cementum-like
tissue growing in continuity with the apical cemental layer of
a molar or premolar that produce expansion of cortical plates
and pain.

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

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
CEMENTOLASTOMA

Osteoblastoma
• Well-demarcated, rounded intraosseous swellings, each with an
active cellular central nidus surrounded by a wide zone of
osteoid, with pain upon palpation.
• 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
Osteoblastoma

Treatment
 local excision or curettage
 A large surgical enbloc removal for large lesions
Prognosis
 Good
 May recur
 Transformation in to osteosarcoma
Osteoblastoma

OSTEOID OSTEOMA
• Benign neoplasm of bone that arises from osteoblasts with similar
clinical, radiographic, and histopathologic features of other two
entities.
Clinical Features:
– mostly before age 25
– more often in males
– Swelling 1-2 cm
– dull or aching pain (relieves with aspirin). Pain due to
intralesional prostaglandins level.
– more in long bone and infrequent in jaws

Sclerotic bone surrounding a radiolucent core
Peripheral radiopacity well demarcated
Treatment
local excision or curettage
Prognosis
Good
Few chances of recurrence

CENTRAL GAINT CELL GRANULOMA
• Central Giant Cell Granuloma (CGCG), 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

• Histologically
• 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.
• The origin of this lesion is unknown but it is assumed to be
triggered by trauma or inflammation.
• 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.

ANUERYSMAL BONE CYST
• Aneurysmal bone cyst (ABC) has been recognized since 1893 when
it was described as an ossifying hematoma by Van Arsdale
• 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

• 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.
• It consists of 5% of all the craniofacial lesions
• Most common in those regions of the skeleton where there is
both a relatively high venous and marrow content.

Conventional /Vascular Solid Mixed
95% (5%) demonstrates features of
both the vascular and solid
types.
rapidly growing
expansive
destructive lesion
A small asymptomatic
lesion
may be a transitory phase of
the lesion because sudden
activation or rapid
enlargement of stable lesions
ha been reported
Causes cortical perforation
and soft tissue invasion.
first noticed as
radiolucency on a routine
radiograph or as a small
swelling.

Hernandez et al. classified ABC as primary and secondary.
a)Primary :-1) Congenital 2) Acquired and could originate from
preexisting A V malformations.
The congenital type is seen in children and young adults with no history
of trauma, whereas the acquired type is found in adults with a history of
trauma.
b)Secondary : associated with degeneration of preexisting lesions such
as a cyst, tumor or fibrosseous lesion. The two lesions could exist
independently.

• 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
• It may appear radiolucent, radiopaque
or mixed.

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 percutaneous
sclerotherapy, diagnostic and therapeutic
embolization, curettage, block resection and
reconstruction, radiotherapy and systemic calcitonin
therapy.
• Self healing cases have also been reported
• Several authors recommend immediate
reconstruction of the defect with autogenous grafts
in cases of esthetic deformity, high risk of fractures
and loss of mandibular continuity.
• Simple curettage recurrence rates varying from 21 to
50%.
• Motamedi et al stated initial resection is not necessary
and have not noted any recurrences following surgical
curettage of mandibular lesions.

OSTEOCHONDROMA
• Osteochondroma or osteocartilaginous
exostosis is characterized by a cartilage-
capped osseous projection protruding
from the surface of the affected bone.
• Although incidence in the general
population is reported as 1%, it may
increase up to 10% with local irradiation
induced osseous changes and up to 23%
with total body irradiation.
• It occurs in adolescence or young
childhood, with 80% of cases being in first
2 decades of life.
• M:F =1:1

• It is considered the most common tumor of the axial skeleton,
comprising 35.8% of benign tumors
• The usual location -distal metaphysis of the femur or the proximal
metaphysis of the tibia
• Various theories of etiopathogenesis have been proposed. However,
most accepted is the Lichtenstein theory, which says that the
periosteum has the potential to develop osteoblasts and chondroblasts
and that osteochondroma develops by metaplastic change in the
periosteum. What triggers this metaplastic change is not known
• Majority of osteochondromas in the craniofacial region occur in
mandibular areas such as the condylar process, coronoid process and
symphysis
Miloro M, Ghali GE, Larsen P, Waite P. Peterson's
principles of oral and maxillofacial surgery; 3rd edition;
volume 1; PMPH-USA; 2004.

• 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
Miloro M, Ghali GE, Larsen P, Waite P. Peterson's principles of oral and
maxillofacial surgery; 3rd edition; volume 1; PMPH-USA; 2004.
OSTEOCHONDROMA

• Histopathology
– Moderatly cellular hyaline cartilage
cap
– Deep layer exhibits endochondral
ossification with deposition of
woven bone on mineralized
cartilage
OSTEOCHONDROMA

Treatment and Prognosis
• Conservative approach /
Surgical Excision
• Recurrence rarely reported
Miloro M, Ghali GE, Larsen P, Waite P. Peterson's principles
of oral and maxillofacial surgery; 3rd edition; volume 1;
PMPH-USA; 2004.

HYPERPARATHYROIDISM
 Primary hyperparathyroidism
 Secondary hyperparathyroidism
 Tertiary hyperparathyroidism
Emkey RD, Emkey GR. Calcium metabolism and correcting calcium deficiencies. Endocrinology and
metabolism clinics of North America. 2012 Sep 30;41(3):527-56.

PRIMARY HYPERPARATHYROIDISM
 Tumor of one of PTH gland.-single adenoma
 Adenomas are located at inferior portion of parathyroid gland
 Mostly seen in women than men & children
 Extreme osteoclastic activity in bones
 Elevates Ca ion conc. in ECF which depresses phosphate ions.

 MEN I (Wermer's syndrome)
consists of hyperparathyroidism and tumors of pituitary and
pancreatic islet cells, often associated with peptic ulcer and
gastric hypersecretion (Zollinger – Ellison syndrome)
 MEN II - carcinoma of the thyroid

Oral Manifestations:
 Dehydration
 Mandibular or maxillary tumors of the bone, which on biopsy
display a brown tumor of von Recklinghausen
 Increased incidence of tori;
 Reduction in cortical bone content leading to osteoporosis

BROWN TUMOR
 Hyperparathyroidism results in
disorders of bone and mineral
metabolism.
 Diffuse and focal lesions may arise
in multiple bones.
 On occasion, a patient
with undiagnosed hyper-
parathyroidism presents with a
lytic lesion that may be mistaken
for a tumor.
 These lesions are termed
"Brown Tumors" due to the
presence of old hemorrhage in
the lesion.

According to Schour and Massler, malocclusion
caused by a sudden drifting with definite spacing of the teeth
may be one of the first signs of the disease.
 Normal trabecular pattern is lost & replaced by granular or
ground glass appearance.
 Moth-eaten like appearance of jaw bones
 Teeth are mobile and migrate.
 Lamina dura diminished or completely absent in 10% of cases.

Osteitis Fibrosa Cystica
 The unique bone involvement in hyperparathyroidism is
osteitis fibrosa cystica.
 In the past osteitis fibrosa cystica occurred in 10 to 25
percent of patients with hyperparathyroidism.
Histologically the pathognomonic features are a reduction in the number of
trabeculae and increase in the giant multinucleated osteoclasts in scalloped
areas on the surface of the bone. (Howship’s lacunae) and a replacement of the
normal cellular and marrow elements by fibrous tissues. Loss of lamina dura of
the teeth is less specific. Tiny “punched out” lesions may be present in the skull,
producing the so called salt and pepper appearance.

Osteitis Fibrosa Cystica

SECONDARY HYPERPARATHYROIDISM
 Vitamin D deficiency
 Chronic renal disease
 Hypocalcemia, hyperphosphatemia & increased serum
alkaline phosphatase

TERTIARY HYPERPARATHYROIDISM
 Parathyroid tumor develop from long standing secondary
hyperparathyroidism.
 Serum calcium is increased
 Phosphorus is normal to increased
 Alkaline phosphatase is increased

TREATMENT
Emergency treatment:
 The solution of IV infusion contains a mixture of mono and
dihydrogen phosphate so that pH is 7.4.
 500ml of this solution should be infused over 4 to 6 hours.
Long term phosphate treatment:
 Oral phosphate is given as diphosphate. Choice depends
upon serum phosphate levels.
 Dose 100 to 300ml per day in divided doses
Phosphate sandoz tablet
 Phosphorous :500mg
 Na: 21 mmol
 K : 3mmol
 Dose: 1 to 6 tab daily

Despite recent advances in the understanding of the natural
history and molecular abnormalities, many questions remain
surrounding the progression and management of Fibro-osseous
lesions. In the absence of comorbidities such as GH excess or
secondary disorders, the expected behaviour of FOL is slow
growing and without functional consequence.
CONCLUSION

In order to optimize patient care, understanding of the
pathophysiologic mechanisms contributing to the various
phenotypes of these condition, as well as the predictors of the
different behaviours of FO lesions, must be improved. The
importance of long-term follow-up of patients with FOL cannot
be overstated because spontaneous recovery is unlikely, and the
course of disease can be unpredictable.
CONCLUSION

Fibro-osseous lesions of jaw

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