BONE DISEASES

OSSIFYING FIBROMA
(Cementifying fibroma / Cemento-ossifying fibroma)

 Ossifying fibroma (OF) is a well circumscribed, sometimes

encapsulated neoplasm composed of fibrous tissue containing
varying amounts of calcified material.
 This calcified material may be bone, cementum or a mixture of
both.
CLINICAL FEATURES: -
Age incidence: 3rd and 4th decades.
Sex incidence: Female to male ratio in
5:1
Site predilection:
 Mandible involved more frequently than
maxilla.
 Within mandible, premolar – molar area is the
commonest site affected.
Signs & symptoms:
 Small lesions are asymptomatic and
detected only during routine
radiographic examination.
 Larger lesions may cause painless
expansion of involved bone.
 Expansion of bone can cause facial
asymmetry.
 Pain and paresthesia are very rarely
noted.
RADIOGRAPHIC FEATURES:
 Most often lesions are well defined,
unilocular.
 Some lesions may be mixed radiopaque-
radiolucent depending on the amount of
calcified material present in the tumor.
 Large lesion may produce root
divergence and root resorption.
HISTOLOGICAL FEATURES:
 Most tumors are well circumscribed
masses composed of fibrous tissue and
containing calcified material.
 The calcified material may be in the
form of irregular trabeculae of osteoid
or basophilic, globular calcifications
resembling cementum.
 Many times both are present in the
same lesion.
 JUVENILE OSSIFYING FIBROMA
 Uncommon lesion of bone.
 Differentiated from ossifying fibroma on the
basis of age incidence, site predilection and
clinical behavior.
 Two patterns recognized – trabecular and
psammomatoid.
CLINICAL FEATURES: -
Age incidence: Patients age 6 months to 70 years
years of age.
Sex incidence: Male
Site predilection:
 Most commonly involves orbital and frontal bones.
 Maxilla is involved more commonly.
Signs & symptoms:
 Most tumors show rapid growth.
 In such cases, pain and paresthesia may
be noted.
 Cortical expansion and facial
asymmetry is seen with jaw lesions.
 Orbital and sinus involvement may
cause exophthalmus, proptosis and nasal
obstruction.
RADIOGRAPHIC FEATURES
 Can be radiolucent or mixed radiopaque-radiolucent
depending on amount of calcified material present within the
tumor.
 Lesion may be well demarcated or lack continuity with
adjacent bone.
PSAMMOMATOID OSSIFYING FIBROMA
HISTOLOGICAL FEATURES: -
1. TRABECULAR J.O.F:
 Both patterns of JOF well
circumscribed but not encapsulated.
 Tumor composed of fibrocellular CT,
areas of hemorrhage and occasional
multinucleated giant cells.
 Mineralized component shows
irregular strands of osteoid lined by
plump osteoblasts.
2. PSAMMOMATOID JOF:
 The stroma is similar to trabecular
JOF.
 The mineralized material is composed
of concentric, lamellated and
spherical ossicles.
 These ossicles vary in size and
typically have basophilic centers with
eosinophilic osteoid rims.
 PAGETS DISEASE
METABOLIC CONDITION
BY DR.UZMA TARIQ

15
 FUNCTION OF BONE

 STRUCTURE
 STRENGTH
 SIZE

16
  Bone are particularly sensitive to physiologic and genetic
derangement result in alteration to their structure and
mineral content.

17
 KEY MOLECULE TO REGULATION
OF BONE CELLS
 Cbfa 1/Run X2,OSX,C-fos,ets-1
causes bone precursurs cells to fully functioning cell i.e
osteoclast and osteoblast
 Osteoprotegerin ,effects osteoclast differentiation
 RANKL,efffects osteoclast differentiation and enhance
osteoblastic activity.

18
 PAGETS DISEASE
 Uncoordinated increase in the osteoclastic and osteoblastic
activity of bone cells of older adults,producing larger but
weaken bone,extensively vascularized with extensive pain.

19
 ETIOLOGY
 Unknown
 Transmitted as autosomal dominant diease.
 Increase in parathyroid hormone.
 Autoimmune connective tissue disease.
 Vascular disorders.
 Viral infection

20
 EPIDIMIOLOGY
 Paget disease of bone affects the skeleton in up to 2% to
3% of population.

21
 PREVALANCE
 ENGLAND
 FRANCE
 GERMANY

22
 PATHOGENESIS

 LYT IC PHASE -osteoclatic activity

 MIXED LYTIC
 SCLEROTIC OR BURNED OUT PHASE

23
 Oral manifestation
 Jaw involvement is common 2:1 max and man
 Maxilla enlargement
 Alveolar ridge become widened
 Palate is flattened
 Teeth –loose-migrate-spacing
 Incompetent lip-enlarged jaw
 Inability to wear denture

24
25
26
  Monostotic or polyostotic
 Dull pain persistant pain
 Bone become warm to touch
 Susceptible to fracture
 Non specific ,headaches,impaired hearing tinnitus
 Hat size increase
 Cranial nerve palsies
 Changes in vision,sycope,incontinence,dementia
 Brainstem compressive syndrome
 Loin like face

27
28
 RADIOGRAPHIC FEATURES

 Multiple,diffuse or isolated
 Osteoporosis circumscripta
 Minute area of variation in radiodensity
 Cotton –wool apprearance
 Loss of normal trabeculae pattern.
 Hypercementosis
 Root resorption
 Hot spot

29
30
RADIOGRAPHIC
FEATURES:
 Early stage (lytic) -
radiolucency and alteration of
trabecular pattern.
 Late stage (osteoblastic) –
patchy areas of sclerotic bone
is formed, called “cotton wool”
appearance.
 Dental radiographs also
show the classical cotton
wool appearance.
 Extensive
hypercementosis can be
noted.
 Laboratory finding
 Inc. alkaline phosphatase level in serum
 Inc. urinary hydroxyproline
 Urinary n-telopeptide
 Alpha-c telopeptide
 Serum calcium normal

34
 Histologic feature
 Initial osteolytic phase _osteoclast(100 nuclei)
 Mosaic pattern with reversal lines.
 Progressive disease osteoblast prodominant
 Marrow spaces filled with highly vascularised connective
tissue.
 Vasodilatation
 Trabeculae pattern lost
 Burned out phase ,decrease mineralization
 Hypercementosis

35
36
37
 Laboratory finding
 Inc. alkaline phosphatase level in serum
 Inc. urinary hydroxyproline
 Urinary n-telopeptide
 Alpha-c telopeptide
 Serum calcium normal

38
 TREATMENT

 Calcitonin
 Bisphosphonates
 Vitamins
 Cytotoxic drugs
 Surgery

39
 ADVERSE EFFECTS
 Due to treatment:

 Bleeding
 Osteomylitis
 Osteonecrosis

40
 COMPLICATIONS

 Osteosarcoma 60% cases

 Fibrosarcoma

41
 OSTEOPETROSIS
(Albers - Schönberg Disease, Marble bone disease)

 Rare hereditary bone disorder characterized by

increase in bone density due to defect in bone
remodeling caused by failure of normal
osteoclast function.
 Two clinical types – infantile and adult
osteopetrosis.
PATHOGENESIS: -
 Osteoclasts fail to function normally.
 As a result, bone remodeling is affected.
 Defective bone resorption combined with
continued bone deposition results in thickening
of cortical bone and sclerosis of cancellous bone.
 I. INFANTILE OSTEOPETROSIS
CLINCAL FEATURES: -
 Autosomal recessive trait.
 Diffusely sclerotic skeleton, marrow failure and signs of cranial
nerve compression present.
 Initial signs – normocytic normochromic anemia and
hepatosplenomegaly.
 Increased susceptibility to infections due to granulocytopenia.
Oral manifestations:
 Facial deformity leading to
hypertelorism, snub nose, frontal
bossing etc.
 Delayed tooth eruption and
osteomyelitis of jaws.
 Sclerosis of skull bones leads to
narrowing of foramina which causes
compression of various cranial nerves –
blindness, deafness, facial paralysis etc
RADIOGRAPHIC FEATURES: -
• Wide spread increase in bone density.
• Distinction between cortical and cancellous
bone is lost.
• Dental X rays – difficult to distinguish roots.
 II. ADULT OSTEOPETROSIS
CLINICAL FEATURES: -
 Discovered late in life – milder symptoms.
 Autosomal dominant trait.
 About 40% cases are asymptomatic.
 Axial skeleton shows sclerosis, while long bones
show little or no defects.
 HISTOLOGICAL FEATURES:

 Several types of abnormal

endosteal formation.
- Tortuous lamellar
trabeculae.
- Globular amorphous
bone.
- Osteophytic bone
formation

48
 REFERENCES
 SHAFER ,8th edition
 Contemprary of oral pathology by MOSBY
 Cawson 8th edition

49
 THANKS

FOR YOUR ATTETION

50
 MCQ’s

1. FIBROUS DYSPLASIA IS A FIBROSSEOUS DISEASE

CHARACTERISZED BY FIBROUS TISSUE REPLEACED
WITH
a) METAPLASTIC BONE FORMATION
b) MALIGNANT BONE FORMATION
c) DYSPLASTIC BONE FORMATION
d) DYSTROPHIC BONE FORMATION
e) NORMAL BONE FORMATION
Key A
2. ‘’CAFÉ AU LAIT” SPOT IS A FEATURE OF TYPE OF
FIBROUS DYSPLASIA.
a) POLYSTOTIC FIBROUS DYSPLASIA
b) MONOSTOTIC FIBROUS DYSPLASIA
c) CHERRUBISM
d) PERIAPICAL CEMENTAL DYSPLASIA
e) BOTH A AND B
Key A
3. FIBROUS DYSPLASIA OF MULTIPLE BONES,
PIGMENTED LESION OF THE SKIN AND ENDOCRINE
DISTURBANCE IS SHOWN IN
a) JAFFE’s TYPE
b) ALBERT’s SYNDROME
c) APERT’S SYNDROME
d) ALBRIGHT’S SYNDROME
e) DOWN SYNDROME
Key D
4. GROUND GLASS/ PEAUD’ ORANGE IS A
RADIOLOGICAL FEATURE OF
a) FIBROUS DYSPLASIA
b) CLEIDO CRANIAL DYSPLASIA
c) OSTEITIS DEFORMANS
d) ECTODERMAL DYSPLASIA
e) MARFAN’S SYNDROME
Key A
5. TYPICAL HISTOLOGICAL FEATURE OF FIBROUS
DYSPLASIA
a) JIGSAW- PUZZLE APPEARANCE
b) “C” SHAPED TRABECULAE
c) STARRY SKY APPEARANCE
d) CART AND WHEEL APPEARANCE
e) NON OF THE ABOVE
Key B
THANK YOU