4. DEFINITION-
The term fibro-osseous lesion (FOL) is a generic designation of a
group of jaw disorders.
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.
Represent diverse group of entities that are characterized by
replacement of normal bone with a variable amounts of
collagenous connective-tissue matrix containing new bone
and/or cementum-like materials.
5. • 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.
Benign mesenchymal skeletal tumors in which mineralized
tissue, blood vessels and giant cells in varying proportions
replace normal bone.
*
Waldron CA.Fibro-osseous lesions of the jaws. JOral Maxillofac Surg. 1985Apr;43(4):249-62.
6. • WHO Classification (1971)
• 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)
• Neville (1995)
• Brannon & Fowler Classification (2001)
• WHO Classification Of Fibro-Osseous Lesions Of Jaws (2005)
• Paul M. Speight & Roman Carlos Classification (2006)
• Eversole Classification (2008)
177-202
*
8. 1. Osteogenic Neoplasms
a. A.Cemento-Ossifying Fibroma (Cementifying Fibroma, Ossifying
Fibroma)
2. Non-Neoplastic Bone Lesions
a. Fiberous Dysplasia Of Jaws
b. Cemento-Osseous Dysplasia
I. Periapical Cemental Dysplasia
II. Florid Cemento-Osseous Dysplasia
III. Other Cemento-Osseous Dysplasia
c. Cherubism (Familial Multilocular Cystic Disease Of The Jaws)
d. Central Giant Cell Granuloma
e. Aneurismal Bone Cyst
f. Solitary Bone Cyst
WHO Classification (1992)
9. WHO(2005)
FIBROUS DYSPLASIA
MONO OSTOTIC
POLY OSTOTIC
CRANIOFACIAL
CEMENTO-OSSEOUS DYSPLASIA
PERIAPICAL
FOCAL
FLORID
FAMILIAL GIGANTIFORM CEMENTOMA
CEMENTO-OSSIFYING FIBROMA
CONVENTIONAL
JUVENILE AGGRESSIVE
TRAUMATIC BONE CYST AND ANEURYSMAL BONE CYST
10. (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
11. 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
12. FIBROUS DYSPLASIA
Term coined by Louis Lichenstein in 1938
First described by Von Recklinghausen in 1891
Is a skeletal developmental anomaly of the bone-forming
mesenchyme that manifests as a defect in osteoblastic
differentiation and maturation.
Characterized by replacement of normal bone, by
excessive proliferation of cellular fibrous connective
tissue intermixed with irregular bony trabeculae.
Normal medullary bone is replaced by abnormal fibrous
connective tissue
13. ETIOPATHOGENESIS
Post zygotic mutation of GNAS1(Guanine nucleotide
binding protein, α-stimulating activity 1) gene
Gs alpha subunit of G protein is a so-called gain of function
mutation resulting in an increased cAMP concentration
Abnormal osteoblast differentiation – abnormal bone
production
Increase IL-6-induced osteoclastic bone resorption
Endocrinal disturbances
14.
15.
16. MARX & STERN
Mutation of gene that encode
for transducer protein required
for bone maturation.
Very early stage mutation –
McCune-Albright Syndrome
Jaffe-Lichtenstein Syndrome
Early stage mutation –
Polysototic FD
Mutation at 6th wk IUL –
Craniofacial FD
Mutation after 6th wk IUL –
Monostotic FD
Clinical severity --- Time of the
mutation
Early embryonic life ---
Undifferentiated stem cells of
osteoblasts, melanocytes, endocrine
cells
Later embryonic development ---
Skeletal progenitor cell,
Postnatal life --- depends of which
bone is affected
17. 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%)
18. CLINICAL FEATURES-
Monostotic- 80-85%
Site – ribs,tibia,femur,craniofacial bones
Age – 2-3rd decade
Most involvement in jaw is monostotic
Maxilla(premolar) > Mandible (Body)
Maxillary lesion may extend to-
zygoma, sphenoid, sinuses, orbit
(CRANIOFACIAL FIBROUS DYSPLASIA)
Characterized by involvement of a single bone
Most commonly diagnosed in young adults with no
sex predilection
Clinical examination reveals a painless swelling or
bulging of the buccal plate.
20. Polyostotic Fibrous Dysplasia (cont.)
There are several asso. syndrome
1.McCune-Albright syndrome -Characterized by endocrine abnormalities,
precocious puberty in females, stunting or deformity of skeletal growth in
both sexes as a result of premature closing of the epiphyseal plates
Café-au-lait spots.
2. Jaffe-Liechtenstein Syndrome –
Polyostotic FD
Cutaneous pigmentation
21. Craniofacial fibrous dysplasia
Involves the maxilla with extension into the sinuses
and adjacent zygoma, frontal,sphenoid,
and occipital bones
Painless enlargement / Facial asymmetry
Skull –
Intracranial Pressure - Limb paresis
Deteriorating vision / hearing
Cranial nerve compression( 2, 3, 4, 6, 7th mostly)
Orbit –
Displacement of eyeball
Proptosis
Deteriorating vision / Diplopia / Blindness
Pain in eye
23. RADIOGRAPHIC APPEARANCE
DIFFUSE MARGINS
NO ROOT RESORPTION, DISPLACEMENT RARE
FUSIFORM EXPANSION OF BONE
GROUND GLASS / PEU’D ORANGE APPEARANCE
Staging according to Mohammadi-Aragi
Pagetoid (56%)
Radiolucent (21%)
Sclerotic (23%)
24. note enlargement of
affected area, buccal
expansion of the
mandible and
granular opacity
“Orange peel appearance of
fine dense
trabeculae seen on intra-oral
radiography
in late stage.
25. FIBROUS DYSPLASIA
Location
More frequent in posterior
Lesions are usually unilateral
Shape and Borders
Usually poorly defined, with the lesion gradually blending into the normal
trabecular
Internal Architecture
Highly variable
Mixed lucent and opaque
Early lesions may be more lucent
Trabeculae are shorter, thinner, more numerous, and irregularly aligned
26. PA projection
O P G
Fibrous Dysplasia
Fibrous dysplasia: Lesion of mandibular
ramus in the intermediate maturation stage
(mixed radiolucency/radiopacity).
27. EFFECT ON ADJACENT STRUCTURES
Superior displacement
of the mandibular
canal
Maxillary lesions
may expand into
the maxillary sinus
Effect on adjacent structures
1. Small lesions are entirely
contained in the bone
2.Teeth may be displaced
5. Lamina dura may be
replaced with
the abnormal bone of the
lesion
6. PDL space may appear
narrowed
28. Effects on adjacent structures
1. Small lesions are entirely contained in the bone
2. Expanded and thinned cortices
3. Maxillary lesions may expand into the maxillary sinus
4. Teeth may be displaced
5. Lamina dura may be replaced with the abnormal bone of the lesion
6. PDL space may appear narrowed
A pathognomonic feature of fibrous dysplasia may be the superior
displacement of the mandibular canal. This is due to the epicenter of the lesion being
below the canal.
30. Alkaline phosphate levels are sometime elevated.
Calcium and Phosphorus are in normal range
Differential Diagnosis
While many other lesions present similar alterations in radiographic
appearance, the patient’s age, unilateral, monostotic lesions, and
painless bony expansion often lead to a diagnosis of fibrous dysplasia
based on the radiographic appearance alone. It is usually confirmed by
histopathological study.
Lesions to be considered include:
Periapical cemental dysplasia
Pagets disease of bone
Osteomyelitis
Osteosarcoma
Cementoossifying fibroma
33. MEDICAL-
Aminobisphosphonate (Pamidronate)
Dose – IV infusion 60mg( children- 1mg/ kg) over 4 hrs
( Series of 3 doses at 1 wk interval repeated at 6months)
Calcium (500-1500mg/day)
Vit. D (800-1200 IU/day)
38. RESULTS –
1. Decreased bone pain
2. Decreased biochemical markers of bone turnover
3. Radiograph – filling of lytic lesion & cortical thickening
DRAWBACKS –
1. No convincing evidence of benefit in children &
adolescents.
2. Not the cure – don’t affect mutated osteoblasts.
3. Bisphosphonate induced osteonecrosis
( NOVARTIS & FDA 2004)
39. ZONE-1 ZONE-2 ZONE-3 ZONE-4a ZONE-
4b
Most
evident
parts on
face-
Frontal
Nasal
Orbit
Zygoma
Ethmoid
Maxilla(upp
er
Hair
covered
cranium -
Occipital
Temporal
(Lat.
Cranial
base)
Difficult/
Dangerous
areas –
Pterygoid
Sphenoid
(Central
cranial
base)
Maxillary
alveolar
bone
Mandible
Radical
excision &
reconstructi
on
Conservativ
e or radical
excision
(optional)
Observatio
n
Surgery if
symptomati
c
Conservativ
e excision
&
recontourin
g
Conservativ
e excision
&
recontourin
g
SURGICAL TREATMENT PROTOCOL BASED ON LOCATION OF LESION
40. Re-growth after surgical shave 25-50%
(C.A.Waldrone,JOMS,1993)
Surgical intervention - after growth completion
SARCOMATOUS TRANSFORMATION -
Less than 1% (Ruggieri,Cancer,1994)
More common in long standing cases of polystotic FD
Radiotherapy contraindicated – risk of malignant transformation (44%)
(C.A.Waldrone,JOMS,1993)
(Bibby K, British J Ophthalmol,1994)
LITERATURE REVIEW
41. CHERUBISM
(FAMILIAL / CONGENITAL FIBROUS DYSPLASIA)
The condition is named due to the facial appearance -plump
cheeked little angels with upward directed look depicted in
renaissance painting.
Autosomal dominant genetic defect
Gene responsible for cherubism – chromosome 4p16.3
(Tiziani V. et al Am J Hum Genet 65(1):158-166 , 1999)
Pathogenesis –
Affect remodeling of bone in the specific anatomic limit of
embryonic mandible and sometimes mandible and maxilla
42. 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
43. Lesion grows slowly but no perforation of cortex.
Marked cervical lymphadenopathy.
Agenesis of 2nd and 3rd molars of mandible.
Premature shedding of deciduous teeth.
Permanent dentition – teeth missing, failure of teeth eruption, teeth
displacement.Severe cases- tooth resorption.
Speech difficulty.
Few cases connected with Noonan’s syndrome- Lesion in the humerus,
gingival fibromatosis, psychomotor retardation, orbital involvement,
obstructive sleep apnea.
47. Histologic Features:
Consists variable
number of
multinucleated giant
cells.
Foci of extravasated
blood are commonly
present.
Cuff like deposits
surrounding small
blood vessels
throughout the lesion.
48. TREATMENT –
Cosmetic recontouring recommended
for esthetics
Self regression after puberty & often complete by 18-20 yrs
Radiograph show only partial bony regeneration as residual
radiolucency
Defer surgery until after puberty – Aesthetic correction
Malignant transformation – no evidences so far
49. CEMENTO‐OSSEOUS
DYSPLASIA
The term cemento‐osseous dysplasia encompasses a spectrum of
dysmorphic bone and cementum
ETIOPATHOGENESIS - ??
mesenchymal stem cells that have lost their
ability to maintain their structural morphology
Classified as —
Periapical osseous dysplasia
Focal osseous dysplasia
Florid osseous dysplasia
50. PERIAPICAL CEMENTAL DYSPLASIA
PCD is a localized change in bone metabolism.
CLINICAL FEATURES
Asymptomatic , Multiple or solitary
Site - Periapical region of lower ant.teeth
Associated teeth – Vital
Usually an incidental radiographic finding
– F:M 9:1
– 3:1 African: Caucasian
– Frequent in Asians
– Mean age = 39 yrs
Radiographic Features
Round, oval or irregular shape
May have a sclerotic border
51. RADIOGRAPHICALLY -
STAGE I -
OSTEOLYTIC STAGE II – CEMENTOBLASTIC STAGE III - MATURE
Well defined peri
apical Radiolucency
Mixed radiolucent &
radioopacity
Radiopaque mass
surrounded by
radioluccent line
– Well defined
– Round, oval or irregular shape
– May have a sclerotic border
52. Effects on adjacent structures
May efface the lamina dura of adjacent teeth
Root resorption is rare
Surrounding bone may become sclerotic
Occasionally, large lesions may cause
expansion of the jaws
TREATMENT - No treatment required
56. FLORID OSSEOUS DYSPLASIA
(Sclerosing Osteitis / Diffuse Chronic Sclerosing
Osteomyelitis / Gigantiform Cementoma)
Called FCOD when lesions are present in three or more
quadrants
Often present in both jaws
More common in mandible
Alter bone pattern in bilateral fashion
Site – Tooth bearing area
Associated teeth - Vital
Specially affect black adult females
Asymptomatic jaw expansion may be noted
60. DEFINITION –
Benign neoplasm of bone in which normal bone
architecture is replaced by a tissue composed of
collagen fibers, fibroblasts with various amounts of
calcified tissue resembling bone and/or cementum.
Etiology : from periodontal tissues.
Ossifying fibroma - predominantly bone.
Cementifying fibroma - predominantly cementum.
WHO 1992 – CEMENTO-OSSIFYING FIBROMA
61. CLINICAL FEATURES –
painless, slow-growing,
and expansile lesion.
Age – 2-4th decade
Sex – Females > males (5:1)
Site – Tooth bearing areas of jaw
(Body of mandible)
Solitary lesions
Causes gradual expansion of cortical plates – Bony hard
swelling
62. Pain , Paresthesia , Perforation ,Mucosal ulceration : Rare
Effects on adjacent structures
Tumor-like behavior:
Concentric growth and expansion
Displaces teeth
Expands and thins cortices
May fill entire maxillary sinus, but retains bony cortex around
lesion.
63. Early lesions Mature lesions
Maturing lesion
RADIOGRAPHICALLY
Sharp border between lesion and normal bone
65. HISTOLOGY
Woven bone with osteoblast rim
Pattern of mineralization varies from
place to place
Variable vascularity
Sometimes encapsulated
well demarcated lesion consisting of a
fibroblastic stroma containing plexiform
and lamellar bone in addition to acellular
mineralized material .
66. TREATMENT –
Surgical enucleation through intraoral approach.
Large lesions – Surgical excision followed by
Bone graft
Recurrence - Rare
Malignant transformation – No evidence so far
70. PAGET’S DISEASE
Synonym: Osteitis deformans
Paget’s disease is named after SIR JAMES PAGET
Paget’s disease is characterised by excessive & abnormal
remodeling of bone. This excessive remodelling gives rise
to bones that are extensively vascularized, weak, enlarged
& deformed with subsequent complications.
71. • ETIOLOGY- Unknown.
• Genetic link, as 7 to 10 fold increase in incidence of the
disease was observed in relatives of patients.
autosomal dominant inheritance.
Paget’s disease is characterised by enhanced resorption of
bone by giant multinucleated osteoclasts with formation of
disorganized woven bone by osteoblasts. Three phases are
seen
- Lytic
- Mixed lytic & blastic
- Sclerotic
72. Clinical features
Age of occurrence
• Prevalence of the disease increases with age.
• Recognised after 50years of age.
Sex predilection
• Male to female ratio is approximately 3:2
73. PAIN-- dull constant aching pain deep below the soft
tissues.
Involved bones become warm to touch because of
increased vascularity.
Changes in vision- secondary to optic nerve involvement
Other typical findings & complaints include :
pathologic fractures
non specific headaches
impaired hearing & tinnitus
74. ORAL MANIFESTATIONS
• Predilection seen for maxilla – 2:1
• The maxilla exhibits progressive
enlargement, alveolar ridge becomes
widened & palate flattened.
75. The teeth present may become loose & migrate,
producing spacing.
As the disease progresses, the mouth may remain
open, exposing the teeth, because the lips are too
small to cover the enlarged jaw.
Edentulous patients with dentures commonly
complain of an inability to wear them because of
increasing tightness due to expansion of the jaw.
76. RADIOGRAPHIC FEATURES
The radiographic features of Paget’s disease are varied & depend on the
stage of the disease encountered.
Osteolytic areas are seen as multiple & diffuse or isolated destructive lesions.
Isolated lesion in skull- osteoporosis circumscripta.
The osteoblastic areas appear as radio opacities with patchy distribution.
This patchiness has been termed a ‘COTTON-WOOL’ appearance.
77. Other findings:
Generalized hypercementosis
Loss of lamina dura
Root resorption has been reported in some
cases, but this is unusual
78. LABORATORY FINDINGS
Serum calcium & phosphorous levels are usually
within normal limits.
Serum alkaline phosphatase level elevated, however
to extreme limits – over 250 Bodansky units.
Urinary hydroxyproline levels are elevated as they
reflect increased osteoclastic activity & bone
resorption.
79. HISTOLOGIC FEATURES
The microscopic appearance of bone varies remarkably, depending
upon the stage of disease encountered.
Repeated formation & removal of bone results in the appearance of
many small irregularly shaped bone fragments that appear to be joined
in a JIGSAW or MOSAIC pattern, with deeply staining hematoxyphilic
reversal lines. This is the histologic hallmark of Paget’s disease
80. Excess production of parathyroidhormone results in the
condition known as hyperparathyroidism.
Primary
Secondary
Hyperparathyroidism
81. PRIMARY HYPERPARATHYROIDISM
Is the uncontrolled production of parathormone (PTH)
usually as a result of a
Parathyroid adenoma (80 to 90 % of cases)
Parathyroid hyperplasia (10 to 15%)
Parathyroid carcinoma (1%)
SECONDARY HYPERPARATHYROIDISM
PTH is continuously produced in response to chronic low levels
of serum calcium, a situation usually associated with chronic
renal disease.
82. AGE OF OCCURRENCE :
Most patients are older age.
SEX PREDILECTION :
Women have this condition 2 to 4 times more often than
men.
CLINICAL FEATURES :
Typically the condition is identified on routine serologic
testing &
majority of patients are relatively asymptomatic.
83. Patients with classic signs & symptoms are described
as having
STONES
BONES
GROANS
MOANS
84. STONES
renal calculi (kidney stones) because of elevated serum
calcium levels.
Metastatic calcifications are also seen frequently
involving other soft tissues, such as blood vessel walls,
subcutaneous soft tissues, the sclera, dura and region
around the joints.
85. BONES
Refers to a variety of osseous changes that may occur
With persistent disease, other osseous lesions develop, such as
BROWN TUMOUR OF HYPERPARATHYOIDISM.
This lesion derives its name from the colour of the tissue
specimen, which is usually a dark red-brown because of the
abundant hemorrhage & hemosiderin deposition within the
tumor.
86. They commonly affect the MANDIBLE, CLAVICLE,
RIBS & PELVIS. They may be solitary but are often
multiple & long standing lesions may produce
significant CORTICAL EXPANSION.
The most severe skeletal manifestation of chronic
hyperparathyroidism is OSTEITIS FIBROSA CYSTICA,
a condition that develops from central degeneration &
fibrosis of long standing brown tumours.
87. GROANS
Refers to the tendency for the development of duodenal
ulcers.
MOANS
Changes in mental status are often seen, ranging from
lethargy &
weakness to confusion or dementia.
88. GENERAL RADIOGRAPHIC FEATURES :
Osteitis fibrosa cystica are localized regions of bone loss produced by
osteoclastic activity, resulting in a loss of all apparent bone structure.
Demineralization & thinning of cortical boundaries often occur in the
jaws such as the inferior border of mandible & the cortical outlines of
the maxillary sinuses
In prominent hyperparathyroidism, the entire calvarium has a
granular
appearance caused by the loss of central trabeculae & thinning
of cortical plates.
89. HISTOPATHOLOGICAL FEATURES :
The brown tumor of hyperparathyroidism is histopathologically
identical to the CGCG of the jaws.
Both lesions are characterized by a proliferation of exceedingly
vascular granulation tissue, which serves as a background for
numerous multinucleated osteoclast type giant cells.
90. TREATMENT
PRIMARY - hyperplastic parathyroid tissue or the functional tumor
must be removed surgically to reduce PTH levels to normal.
SECONDARY - signs and symptoms related to renal calculi.
Restriction of dietary phosphate & use of phosphate-binding agents.
PARATHYROIDECTOMY
91. ANEURYSMAL BONE
CYST
First described by Jaffe & Lichtenstein in 1942.
First case in jaw was reported by Bhaskar & Bernier in 1958.
Is a non-neoplastic, reactive lesion of bone generally
consisting of several cavities filled with blood and deprived
of an endothelial lining.
Not a true cyst, sinusoidal blood filled space.
92. Clinical Features:
Occurs below 20 yrs of age.
No significant predilection
Mandible > maxilla, molar region is the most common site.
History of traumatic injury preceding development
Slow growing, may expand the cortical plate, does not destroy
them.
Aggressive lesions may perforate cortical plate with soft tissue
extension.
93. Teeth may be tender, missing or displaced.
Pain is occasional complaint.
On palpation: egg-shell crackling, non-pulsatile.
On surgical exploration: ‘welling up’ of blood.
Gross examination: blood-soaked sponge.
94. Etiology:
Cause is controversial, the theories put forth regarding the pathogenesis of the lesion are:
- Trauma
- Local changes in hemodynamics
- Reparative response to the hematoma
Phases of pathogenesis:
Initial- osteolytic phase
Active growth phase- rapid destruction of bone, sub periosteal blow out pattern
Mature stage(Stage of stabilization)- formation of distinct peripheral bony shell & internal bony
septa and trabeculae that produce classic soap bubble appearance
Healing phase- progressive e calcification and ossification of cyst- transformation to dense bony
irregular structure
95.
96. Histologic
Features:
Consist of a fibrous
connective tissue stroma
containing of many
sinusoidal blood filled
spaces.
Fibroblast are numerous as
well multinucleated giant
cells.
Vascular spaces lack any
endothelial lining, and giant
cells form part of their walls.
