Fibro-osseous lesions- a thorough extensive Post-graduate level presentation

1. FIBRO-OSSEOUS LESIONS
Aishwarya S Nair
1

2. FIBRO-OSSEOUS LESIONS
-Contents
Introduction
Classification
Importance of Radiology in the Diagnosis of
FOLs
Fibrous Dysplasia
Cemento-osseous Dysplasia
2

3. INTRODUCTION…
3

4. INTRODUCTION…
Fibro-osseous lesions of the jaw comprise a
diverse group of conditions in which the
normal architecture of bone is replaced by
fibrous tissue and may contain varying
amounts of mineralised substance which
may be bone, cementum or both in
appearance.
The term fibro-osseous lesion was originally a
histo-pathological term
4

5. CLASSIFICATION OF FIBRO-OSSEOUS
LESIONS
5

6. CLASSIFICATION SYSTEMS…
The various classifications systems proposed by authors
are enumerated as below.
Charles Waldron Classification Of 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)
6

7. CLASSIFICATION SYSTEMS…
The various classifications systems proposed by
authors are enumerated as below.
Brannon & Fowler Classification (2001)
WHO Classification Of Fibro-Osseous Lesions Of Jaws (2005)
Paul M. Speight & Roman Carlos Classification (2006)
Eversole Classification (2008)
7

8. CLASSIFICATION…
Fibro-osseous Lesions of Jaws Classification
1. Fibrous Dysplasia
a. Monostotic
b. Polyostotic
8
2. Cemento-osseous Dysplasia
a. Periapical Cemental Dysplasia
b. Focal Cemento-osseous Dysplasia
c. Florid Cemento-osseous Dysplasia (Gigantiform Cementoma)

9. CLASSIFICATION…
9
3. Familial Gigantiform Cementoma
6. Miscellaneous
Osteoblastoma, Osteiod osteoma, Cementoblastoma
5. Juvenile Ossifying Fibroma
4. Ossifying Fibroma

10. CLASSIFICATION…
Waldron’s Classification (1993)
1. Fibrous Dysplasia
a. Monostotic
b. Polyostotic
10
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 Cemento-osseous Dysplasia (Gigantiform Cementoma)
d. Ossifying & Cementifying Fibroma

11. CLASSIFICATION…
Waldron’s Classification (1993)
11
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

12. CLASSIFICATION…
Brannon & Fowler’s Classification
1. Fibrous Dysplasia
a. Monostotic
b. Polyostotic
c. Craniofacial
d. McCune-Albright syndrome
2. Osseous Dysplasia
a. Periapical
b. Focal
c. Florid
d. Familial gigantiform cementoma
3. Ossifying Fibroma & Juvenile Ossifying Fibroma
12

13. IMPORTANCE OF RADIOLOGY IN
DIAGNOSIS OF FIBRO-OSSEOUS
LESIONS…
The radiologist plays a central role in diagnosis of
fibro-osseous lesions.
Charles Waldron had stated
“In the absence of good clinical & radiologic
information, a pathologist can only state a biopsy to be
consistent with a fibro-osseous lesion”
Therefore, identification of majority of FOLs is based
on clinical and radiological features.
13

14. IMPORTANCE OF RADIOLOGY IN
DIAGNOSIS OF FIBRO-OSSEOUS
LESIONS…
Many fibro-osseous lesions are symptomless and
require no surgery. Therefore, diagnosis of the
lesions on clinical & radiological features may
obviate the need for surgery
This my benefit the patient, as fibrous dysplasia
may show exaggerated growth after surgery
14

15. IMPORTANCE OF RADIOLOGY IN
DIAGNOSIS OF FIBRO-OSSEOUS
LESIONS…
Radiological evaluation-
Plain radiography
IOPAR
Occlusal radiograph
Bitewing radiograph
15

16. IMPORTANCE OF RADIOLOGY IN
DIAGNOSIS OF FIBRO-OSSEOUS
LESIONS…
Radiological evaluation-
CT- expansile, destructive lesions, cortical
breakthrough & extra-osseous extensions
MRI- solid from non-solid masses
16

17. FIBROUS DYSPLASIA
17

18. FIBROUS DYSPLASIA
-Introduction
WHO (1992) defined fibrous dysplasia as
“A benign lesion, presumably developmental in nature,
characterised by a presence of fibrous connective tissue
with a characteristic whorled pattern and containing
trabeculae of immature bone”
Asymptomatic regional alteration of bone in which normal
architecture of bone is replaced by trabeculae-like osseous
structures; lesions may be monostotic or polyostotic, with or
without associated endocrinal disturbances
- Eversole
18

19. FIBROUS DYSPLASIA
-Introduction
Congenital, skeletal disorder wherein bone is
formed and the maturation is arrested in the
“woven bone stage”.
NON-HEREDITARY DISORDER
19

20. FIBROUS DYSPLASIA
-History
First citation by von Recklinghausen in 1891
Albright pointed out that 2 cases mentioned by von
Recklinghausen- fibrous dysplasia
Lichtenstein- term FIBROUS DYSPLASIA (1938)
Initially considered- Polyostotic
Lichtenstein & Jaffe expanded the concept-
monostotic
20

21. FIBROUS DYSPLASIA
-History
McCune & Albright (1936 &1937) described an
association of abnormal skin pigmentation &
precocious puberty
(McCune-Albright Syndrome)
Jaffe-Lichtenstein described the association of
polyostotic fibrous dysplasia with abnormal
pigmentation
(Jaffe-Lichtenstein Syndrome)
21

22. FIBROUS DYSPLASIA
-Aetiology & Pathogenesis
GNAS1 gene
Activation of GS𝞪 subunit of G protein complex
Production of cAMP
22
MUTATION
CONTINUOUS

23. FIBROUS DYSPLASIA
-Pathogenesis
1. Hyperfunction of endocrine glands
2. Increased production of melanocytes
3. Affects differentiation of osteoblasts— impairs
ability to form mature osteoblasts
Fibrous tissue is produced + trabeculae contain
“fluid-filled cysts” — GROUND-GLASS APPEARANCE
23

24. FIBROUS DYSPLASIA
-Pathogenesis
Undifferentiated stem cells—
Melanocyte progenitor cells
Skeletal progenitor cells
Endocrinal progenitor cells
24

25. FIBROUS DYSPLASIA
-Pathogenesis
Skeletal progenitor cells— Migrate to form
different bones
Multiple bones
Post natal mutation—
Single bone is affected
25

26. FIBROUS DYSPLASIA
-Classification
Monostotic—
Juvenile
Juvenile, aggressive
Adult
26
Polyostotic—
Craniofacial
M c C u n e - A l b r i g h t
Syndrome
Ja f f e - L i ch t e n s t e i n
Syndrome

27. JUVENILE FIBROUS DYSPLASIA
-Clinical Features
AGE- Early to late childhood
SITE- Maxilla > Mandible
27

28. JUVENILE FIBROUS DYSPLASIA
-Clinical Presentation
Asymptomatic
Swelling is not prominent &
eventually becomes
prominent
In teenage— swelling may
become prominent.
Slowly growing ; in
aggressive form, growth is
more rapid than rest of the
bone
28

29. JUVENILE FIBROUS DYSPLASIA
-Clinical Presentation
May cause-
Displacement, rotation or
malalignment of teeth
Malocclusion
Facial deformity
29

30. JUVENILE FIBROUS DYSPLASIA
-Clinical Presentation
Aggressive form:
Pain, trauma & ulceration
2o to impingement by
teeth
In maxilla- may extend to
involve
Floor of the orbit
Nasal passages
30

31. ADULT MONOSTOTIC FIBROUS DYSPLASIA
-Introduction
Rare form
Spontaneously occurring
May appear similar to ossifying fibroma but must be
differentiated from it
31

32. ADULT MONOSTOTIC FIBROUS DYSPLASIA
-Clinical Presentation
Asymptomatic
Diffuse expansion of
cortices
Movement of teeth
within the area may
occur
32

33. ADULT MONOSTOTIC FIBROUS DYSPLASIA
-Radiographic Features
Differs from juvenile
form.
Less homogenous &
p r e s e n t s a s m i x e d
r a d i o l u c e n t - r a d i o -
o p a q u e l e s i o n —
“ COTTON-BALL” pattern
33

34. POLYOSTOTIC FIBROUS DYSPLASIA
-Introduction
Three types under this sub-heading-
Craniofacial form
McCune-Albright Syndrome
Jaffe- Lichtenstein Syndrome
34

35. POLYOSTOTIC FIBROUS DYSPLASIA
-Clinical Features
15-20% of remaining cases- POLYOSTOTIC in nature
SITE- Skull & facial bones, pelvis, femur, tibia, spine &
shoulder girdle;
Single limb or all the limbs with/ without axial skeleton
involvement
UNILATERAL, but if disease is generalised— may be BILATERAL
35

36. POLYOSTOTIC FIBROUS DYSPLASIA
-Clinical Presentation
Symptomatic before 10years
INITIAL PHASE- Pain in limb with
associated limp or spontaneous
fracture
Leg length discrepancy- 70%
cases
Weight-bearing bones— BOWED
Curvature of femoral shaft &
neck- SHEPHERD’S CROOK
DEFORMITY (characteristic feature)
36

37. POLYOSTOTIC FIBROUS DYSPLASIA
-Cafe au lait Macules
Pigmentation present in polyostotic
fibrous dyspalsia -Café au lait
pigmentation
Café au lait pigmentation(50%) –
irregular margins resembling coast
line of Maine ( Neurofibromatosis –
coastline of California)
Café au lait spots is ipsilateral to side
of bony lesion (difference from
neurofibromatosis)
Pigmentation may occur at birth and
may precede other symptoms
37

38. JAFFE-LICHTENSTEIN SYNDROME
-Clinical Features
Variable number of bones are involved
Accompanied by abnormal pigmentation in skin (CAFE-AU-
LAIT SPOTS)
Pigmentation may occur at birth & may precede other
symptoms
Occurs in same side as that of the bony lesion
38

39. McCUNE-ALBRIGHT’S SYNDROME
-Clinical Features
Severe form
Nearly all bones are involved
Endocrine disturbances
Abnormal pigmentation in skin (CAFE-AU-LAIT SPOTS)
39

40. McCUNE-ALBRIGHT’S SYNDROME
-Clinical Features
McCune Albright syndrome affects females >males
The most common endocrinal abnormality: sexual precocity
Other endocrinal manifestations are:
Accelerated skeletal growth Acromegaly
Gigantism Hyperprolactenimia
Cushing’s syndrome Hyperthyroidism
Diabetes mellitus Hypothalamic hypogonadism
Hypophospahtemic rickets Gynecomastia
Spermatogenesis
40

41. CRANIOFACIAL FORM
-Clinical Features
Occurs in 50% of
polyostotic cases;
10-25% of monostotic
cases
SITE- Frontal, sphenoid,
maxillary, ethmoid; less
commonly occipital,
temporal

42. CRANIOFACIAL FORM
-Clinical Presentation
SPHENOID WING & TEMPORAL BONE-
Vestibular dysfunction
Tinnitus
Hearing loss
42

43. CRANIOFACIAL FORM
-Clinical Presentation
ORBITAL & PERIORBITAL REGION-
Hypertelorism
Visual impairment
Exophthalmos
Cranial asymmetry
Facial asymmetry
CRIBRIFORM PLATE-
Hyposmia or anosmia
43

44. FIBROUS DYSPLASIA
-Mazabraud’s Syndrome
44
Rare disease
Association of fibrous dysplasia and intramuscular
myxoma
Greater risk of malignant transformation in fibrous
dysplasia

45. FIBROUS DYSPLASIA
-Radiographic Features
Course of the disease—
Early lesion-
Radiolucent
Ill-defined borders
Surrounding areas-
increased density (granular
appearance)
Unilocular; may appear
multilocular
45

46. FIBROUS DYSPLASIA
-Radiographic Features
Course of the disease—
Mature lesion-
Mixed radiolucent-
radio-opaque lesion
NEW BONE: small radio-
opacities
46

47. FIBROUS DYSPLASIA
-Radiographic Features
Location-
Maxilla: mandible =
2:1
Posterior regions of
jaw
Unilateral (very rarely
extensive lesions may
be bilateral)
47

48. FIBROUS DYSPLASIA
-Radiographic Features
Periphery-
Ill-defined
Gradual blending of
irregular trabeculae &
normal trabeculae
OCCASIONALLY, may
appear sharp & well-
corticated in younger
individuals
48

49. FIBROUS DYSPLASIA
-Radiographic Features
Internal Structure-
Density & trabecular pattern
vary
Variation is more in mandible
May be
Radiolucent,
Radio-opaque or a
Mixed radiolucent-radio-
opaque lesion
49

50. FIBROUS DYSPLASIA
-Radiographic Features
Internal Structure-
OBISESAN ET AL’S CLASSIFICATION of
Radiographic Features of Fibrous
Dysplasia:-
PEAU DE ORANGE- resembling
surface of an orange
GROUND-GLASS- resembling
shattered wind-screen
PAGETOID- wispy arrangement
with alternating areas of
radiolucency & radio-opacity
50

51. FIBROUS DYSPLASIA
-Radiographic Features
Internal Structure-
OBISESAN ET AL’S CLASSIFICATION of
Radiographic Features of Fibrous
Dysplasia:-
FINGERPRINT— swirling pattern
CYST-LIKE RADIOLUCENCY—
Radiolucent lesions resembling
cysts may occur in mature lesions
CHALKY-TYPE— Well-circumscribed
lesion with amorphous dense
radio-opaque material
51

52. FIBROUS DYSPLASIA
-Radiographic Features
Effects on Surrounding
Structures-
Small lesions- No effect
(SUB-CLINICAL VARIETY)
BONE-
Expansion
Maintenance of
thinned outer cortex
52

53. FIBROUS DYSPLASIA
-Radiographic Features
Effects on Surrounding
Structures-
MAXILLARY SINUS-
May expand into it
Displaces cortical outline
Occupies part or most of the
sinus cavity
Extension into sinus is
through LATERAL WALL
53

54. FIBROUS DYSPLASIA
-Radiographic Features
Effects on Surrounding
Structures-
MAXILLARY SINUS-
Last section to be involved:
POSTERO-SUPERIOR PORTION
Parallel thickening of cortical
border-results in residual air
space— approximately the
normal anatomic shape of
the antrum
54

55. FIBROUS DYSPLASIA
-Radiographic Features
Effects on Surrounding
Structures-
ALVEOLAR BONE-
Bone is altered without
affecting the dentition.
TEETH-
LAMINA DURA IS ABSENT
PDL SPACE may appear very
narrow— if fibrous dysplasia
increases bone density.
55

56. FIBROUS DYSPLASIA
-Radiographic Features
Effects on Surrounding
Structures-
TEETH-
Displaces teeth
Interfere with normal
eruption
RARELY, root resorption
may occur
Involved teeth may
have- HYPERCEMENTOSIS
56

57. FIBROUS DYSPLASIA
-Radiographic Features
Effects on Surrounding Structures-
MANDIBULAR CANAL-
Unique in its ability to displace the canal SUPERIORLY 57

58. FIBROUS DYSPLASIA
-Other Imaging Modalities
58
ROLE OF ULTRASOUND:
Helpful for assessing extraskeletal manifestations
Usually USG of thyroid and gonads done : evaluate
activity and structure of glands and gonads
ROLE OF MRI:
Not useful as CT and plain films. On T1-weighted
MRIs, the lesion has low-to-intermediate signal
intensity equal to that of muscle. T2-weighted images
also show low signal intensity owing to the high
content of collagen and bone

59. FIBROUS DYSPLASIA
-Other Imaging Modalities
59
BONE SCINTIGRAPHY:
Accumulation of isotope increases because of the
lesion's hypervascularity.
Pathologic or stress fractures also can increase
isotopic activity in the lesions.
The features on the bone scan are nonspecific for a
conclusive diagnosis based solely on the distribution
of the isotope.

60. FIBROUS DYSPLASIA
-Other Imaging Modalities
BONE SCINTIGRAPHY:
Hot spots or increased
uptake of the
radioisotope tracer
technetium-99m
methylene
diphosphonate (99m Tc
MDP) occurs in the
spine, pelvis, ribs, and
appendicular skeleton.

61. FIBROUS DYSPLASIA
-Differential Diagnosis
Metabolic Bone disease FD
Number of bones
involved
Polyostotic Monostotic
Bilateral Unilateral
Expansion NO ✔
61
Paget’s Disease FD
Expansion ✔ ✔
Age >40 years Younger individuals
Site
If mandible- whole of
mandible
Unilateral

62. FIBROUS DYSPLASIA
-Differential Diagnosis
Periapical Cemental
Dysplasia
FD
Age Older individuals Younger
Bilateral Unilateral
62
Osteomyelitis FD
Expansion
Enlargement- on the
surface of outer cortex
Evidence of original cortex
Expands internal
structure
Sequestrum ✔ NO

63. FIBROUS DYSPLASIA
-Differential Diagnosis
Cemento-ossifying Fibroma FD
Displacement ✔ NO
One specific centre NO ✔
Bone alteration
around teeth
✔ ✔
63
Neoplasm FD
Expansion
Convex extension is
noted
Extension into antrum
causes expansion- but,
original contour is
maintained

64. FIBROUS DYSPLASIA
-Histopathological Features
Woven bone is present in the
form of irregular shaped
trabeculae (Chinese script
writing)
Trabeculae are delicate and are
not connected to one another;
not sharply defined
Bone formed is metaplastic in
nature. This form of metaplasia
is called a fibro-osseous
metaplasia
64

65. FIBROUS DYSPLASIA
-Histopathological Features
Fibrous stroma comprises
immature appearing small,
slender spindle cells in loose
and whorled arrangement.
Giant cells are usually not seen
in lesions of fibrous dysplasia
b u t i f s e e n a r e u s u a l l y
associated with the pre-existing
mineralized tissue.
65

66. FIBROUS DYSPLASIA
-Laboratory Investigations
Serum Ca2+ & P : normal
Serum Alk Phosphatase: ed
Urinary hydroxyproline, specific index of bone
collagen resorption: ed
McCune-Albright’s Syndrome- circulating hormones:
ed

67. FIBROUS DYSPLASIA
-Management
67
Mostly clinical & radiological features are
sufficient to make a diagnosis of Fibrous dysplasia
Reports- exaggeration of growth of lesion— due to
surgical intervention
Monitor the lesion
Ask patient to report if any changes

68. FIBROUS DYSPLASIA
-Medical Management
68
Limited use in polyostotic cases
1. Bisphosphonates: Palmidronate 180mg i.v. every
6months/ i.v. 1-1.5mg/kg/day for 3 consecutive days,
given every 4months
➡es- bone pain
➡es- bone resorption
es- bone mineral density

69. FIBROUS DYSPLASIA
-Medical Management
69
2. Calcitonin: Calcitonin injections in doses ranging
from 50 to100 IU three times weekly for a period of
3-months were given subcutaneously
➡es- bleeding
es- bone formation
3. Supportive therapy: Vitamin D and calcium

70. FIBROUS DYSPLASIA
-Pain in fibrous dysplasia
70
Sprouting of neuroma like structures
As a result of GNAS mutation—increased IL-6 secretion

71. FIBROUS DYSPLASIA
-Pain in fibrous dysplasia
71

72. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
72

73. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Introduction
Localised change in normal bone metabolism
that results in replacement of the components of
normal cancellous bone with fibrous tissue and
cementum-like material, abnormal bone or a
mixture of the two.
Lesion located near the apex of the tooth
73

74. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Clinical Features
AGE- Middle age; >30years and mean age is 39years
GENDER- ♀ : ♂ = 9 : 1
RACE- Blacks : Whites= 3 : 1 ; frequently seen in
Asians
74

75. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Clinical Presentation
VITAL teeth
No h/o pain, sensitivity
Incidental finding
May become very large— expansion of bone— slow
growth

76. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Radiographic Features
Location-
Epicentre- at the apex,
apical-third of root
Mandibular anterior
teeth
Rarely maxillary teeth
Multiple or bilateral;
occasionally solitary
lesion may arise
76

77. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Radiographic Features
Periphery-
Well-defined
Radiolucent border may
surround the lesion
Followed by reactive
sclerotic border
Shape-
May be irregular or have
round or oval shape
77

78. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Radiographic Features
Internal Structure-
Varies depending on
maturity of lesion
EARLY STAGE: Bone is
resorbed & continuous
with PDL space
78

79. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Radiographic Features
Internal Structure-
MIXED STAGE: Radio-
opaque tissue appears
in the lesion. Round/
o v a l / i r r e g u l a r .
Sometimes cementum-
like material may form
s w i r l i n g p a t t e r n
(CEMENTICLES)
79

80. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Radiographic Features
Internal Structure-
MATURE STAGE:
Totally radio-opaque.
Thin radiolucent rim
80

81. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Radiographic Features
Effects on surrounding
structures-
LAMINA DURA: Loss
PDL SPACE: Widened
TOOTH: Not affected; rarely
r e s o r p t i o n o r
hypercementosis
JAW BONE: Expansion may
be seen with thin intact
cortex. Undulating in shape
81

82. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Differential Diagnosis
EARLY STAGE
PCOD
Apical periodontitis/PA
abscess
Pulp vitality test Responsive Not/delayed response
82
MIXED STAGE
PCOD
Rarefying & condensing
osteitis
Apical region Lamina dura intact
Radiolucent zone next to
root apex

83. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Differential Diagnosis
83
MIXED STAGE
PCOD
Rarefying &
condensing
osteitis
Chronic
Osteomyelitis
Apical
region
Lamina dura
intact
Radiolucent zone
next to root apex
Radiolucent zone
next to root apex
Well-defined
lesion
Moth-eaten
appearance
PCOD Calcifying crowns
Age > 30years < 20years

84. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Differential Diagnosis
84
MIXED STAGE
PCOD Odontoma
Radio-opacity Less radio-opaque More radio-opaque
Position irt adj.
teeth
At the apical region
Seldom below; usually
above uneruted teeth
PCOD Post-surgical defect
No such history H/o recent enucleation

85. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Differential Diagnosis
85
MIXED STAGE
PCOD
Osteogenic sarcoma/
Chondrosarcoma/ Metastatic
osteoblastic carcinoma
Rate of
growth
Slowly growing Rapidly growing
Margins Well-defined Irregular
Root
resorption
Rarely May be noted

86. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Differential Diagnosis
86
MIXED STAGE
PCOD Fibrous Dysplasia
Age >30years 1st to 2nd decade
Gender ♀: ♂ = 3:1 No gender predilection
Site Mandible (90%) Maxilla
Jaw
expansion
Nodular/dome
shaped
Fusiform shaped
Frequency of
occurrence
Less common More common
Margins Well-defined lesion
Poorly defined
Imperceptibly merges with adj
bone

87. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Differential Diagnosis
87
MIXED STAGE
PCOD Cemento-ossifying fibroma
Age >30years <30years
Gender Marked ♀
predilection
Lesser ♀ predilection
Site Mandibular anterior PM-M region
Jaw
expansion
Minimal Tendency for expansion
Frequency of
occurrence
Maximum size- < 1cm More common

88. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
-Management
88
Surgical intervention is not indicated

89. FOCAL CEMENTO-OSSEOUS DYSPLASIA
89

90. FOCAL CEMENTO-OSSEOUS DYSPLASIA
-Introduction
The term Focal Cemento-osseous Dysplasia was
first used by Tomich and Summerlin in 1989
90

91. FOCAL CEMENTO-OSSEOUS DYSPLASIA
-Clinical Features
AGE- 4th to 5th decade
GENDER- Female predilection
SITE- Edentulous posterior areas of the mandible
91

92. FOCAL CEMENTO-OSSEOUS DYSPLASIA
-Clinical Presentation
Asymptomatic; no swelling unless it is an old lesion-
has caused expansion

93. FOCAL CEMENTO-OSSEOUS DYSPLASIA
-Radiographic Features
Well-demarcated, mixed
radiolucent-radiopaque lesion
<2cm in size
93

94. FOCAL CEMENTO-OSSEOUS DYSPLASIA
-Histopathological Features
Cellular fibrous tissue—
c o n t a i n s i r r e g u l a r
trabeculae of woven bone
o r c e m e n t u m - l i k e
calcifications
S c a t t e r e d f o c i o f
multinucleated giant cells
may be seen
94

95. FOCAL CEMENTO-OSSEOUS DYSPLASIA
-Management
95
Lesion shows NO tendency to recur
Partial removal- large lesions (doesn’t recur)

96. FIBRO-OSSEOUS LESIONS
Aishwarya S Nair
96

97. FIBRO-OSSEOUS LESIONS
-Contents
Florid Osseous Dysplasia
Gigantiform Cementoma
Cementifying, Cemento-osseous, Ossifying fibroma
Juvenile Ossifying Fibroma
Diagnostic Clues
Conclusion
97

98. FLORID CEMENTO-OSSEOUS DYSPLASIA
98

99. FLORID CEMENTO-OSSEOUS DYSPLASIA
-Introduction
Also called Familial Multiple Cementoma
The term was introduced by Melrose et al in 1976
It is a widespread form of Periapical Cemento-osseous
Dysplasia
Diagnosis-
PCOD in 3-4 quadrants (or)
Extensively formed in one jaw
99

100. FLORID CEMENTO-OSSEOUS DYSPLASIA
-Aetiology
Aetiological factor unknown
Waldron et al have proposed that reactive or
dysplastic changes in PDL may trigger the disease.
100

101. FLORID CEMENTO-OSSEOUS DYSPLASIA
-Clinical Features
AGE- middle age (mean age= 42years)
GENDER- Female predilection; uncommon in males
SITE-
Bilaterally occurring and symmetrical lesions in
jaws
Limited to alveolar bone of the jaws
101

102. FLORID CEMENTO-OSSEOUS DYSPLASIA
-Clinical Presentation
Signs & symptoms are
generally absent
Partially or completely
edentulous
DULL PAIN- if exposed to
oral cavity— 2o infected—
OSTEOMYELITIS
S u p e r fi c i a l - a s b o n e
resorption is faster than
the sclerotic masses
102

103. FLORID CEMENTO-OSSEOUS DYSPLASIA
-Radiographic Features
Location-
Bilaterally present
Both jaws; if only one-
mand>> max
Epicentre- Apical to teeth
within alveolar process
Posterior to canines
Mandible- above the
mandibular canal
103

104. FLORID CEMENTO-OSSEOUS DYSPLASIA
-Radiographic Features
Periphery-
Sclerotic border
In mature lesions, radiolucent, soft-tissue capsule may NOT be
appreciable
104

105. FLORID CEMENTO-OSSEOUS DYSPLASIA
-Radiographic Features
Internal Structure-
Varies from mixed radiolucent-radio-opaque to completely
radio-opaque
Some prominent radiolucent areas— development of simple
bone cyst
Small, oval/round regions to large amorphous calcified areas
105

106. FLORID CEMENTO-OSSEOUS DYSPLASIA
-Radiographic Features
Effect on Surrounding Structures-
Mandibular canal: can be displaced
inferiorly
Maxillary sinus: can be displaced
superiorly
Alveolar bone: expansion of
cortical plates
Roots: May show hypercementosis;
may fuse with cementum of
abnormal tissue of lesion 106

107. FLORID CEMENTO-OSSEOUS DYSPLASIA
-Differential Diagnosis
107
Florid Cemento—osseous
Dysplasia
Paget’s Disease
Mandibular
canal
Above mandibular canal Entire mandible
Site Only jaws
Polyostotic including
jaws
Florid Cemento—
osseous Dysplasia
Chronic Sclerosing
Osteomyelitis
Only alveolar bone Alveolar & basal bone
Cementum-like masses may resemble sequestrum

108. FLORID CEMENTO-OSSEOUS DYSPLASIA
-Management
108
No T/t required
No age limit for cessation of growth
Propensity for 2o infection— oral hygiene must be
maintained

109. GIGANTIFORM CEMENTOMA
109

110. GIGANTIFORM CEMENTOMA
-Introduction
Rare, benign fibro-osseous disease of the jaws
Characterised by formation of massive sclerotic masses of
disorganised mineralised tissue
Norberg in 1931, first described Gigantiform Cementoma
Familial cases > Sporadic cases; autosomal dominant
WHO in 1971, later re-classified it under cemental
lesions
110

111. GIGANTIFORM CEMENTOMA
-Clinical Features
AGE- Younger age group; occasionally older
individuals
GENDER- No gender predilection
SITE- Both the jaws; multifocal & multiquadrant
Lesions tend to plateau after cessation of growth
111

112. GIGANTIFORM CEMENTOMA
-Clinical Presentation
Painless swelling
Expansile lesions
Tooth impaction,
malpositioning of teeth,
malocclusion
Enlargement stops in 5th
decade
112

113. GIGANTIFORM CEMENTOMA
-Radiographic Features
Number- Multiple
Location- Maxilla or mandible
Periphery- Well-defined, well-
circumscribed
Internal Structure- Lobulated,
radio-opaque-radiolucent lesions
113

114. GIGANTIFORM CEMENTOMA
-Radiographic Features
Other features-
Expansile
C r o s s e s m i d l i n e
(sometimes lesion may
develop in posterior
r e g i o n & e n l a r g e
towards the anterior
part of the jaws &
become confluent
114

115. GIGANTIFORM CEMENTOMA
-Management
115
Surgical removal
Incomplete- recurrence
Complete + conservative approach— ideal
approach

116. OSSIFYING FIBROMA
116

117. OSSIFYING FIBROMA
-Introduction
Before refining the concept of focal cemento-
osseous dysplasia, Ossifying fibroma, was thought
to be common
Presently— relatively rare
Radiographically- focal cemento-osseous dysplasia
Histopathologically- neoplasm with significant
growth potential
117

118. OSSIFYING FIBROMA
-Introduction
In 1972, WHO classification separated
cementifying and ossifying fibroma
Cementifying fibroma- spherical calcifications
Ossifying fibroma- predominant osseous
component
118

119. OSSIFYING FIBROMA
-Introduction
Origin of these cementum-like calcifications—
uncertain— extra-gnathic sites
Bone & cementum— cannot be distinguished
histologically
119

120. OSSIFYING FIBROMA
-Aetiology & Pathogenesis
Occurs mostly in jaws— originate from pluripotent cells-
periodontal membrane
Recently mutations in HRPT2 gene— rare syndrome called
Hyperparathyroidism- jaw tumour syndrome
Characterised by
Parathyroid adenoma/carcinoma
Ossifying fibromas of the jaws
Renal cysts
Wilm’s tumour
120

121. OSSIFYING FIBROMA
-Aetiology & Pathogenesis
Identification of HRPT2 gene mutation— 2
sporadic cases
Function of parafibromin & mechanism of
tumour formation— NOT KNOWN
121

122. OSSIFYING FIBROMA
-Clinical Features
AGE- 3rd- 4th decade of life
GENDER- Female predilection
SITE- Mandible>> Maxilla
Premolar-molar region
Tooth-bearing areas— may extend into ramus
122

123. OSSIFYING FIBROMA
-Clinical Presentation
Hard, localised, slow-growing
painless mass
May displace adjacent structures
Exfoliation of teeth
Expansion of inferior border
followed by buccal cortical plate
expansion
123

124. OSSIFYING FIBROMA
-Gross Appearance
Hypovascular
Well-demarcated— easy
separation from
surrounding bone
124

125. OSSIFYING FIBROMA
-Radiographic Features
Number- Single
Location- Exclusively in
facial bones; mandible-
PM-M regions; superior
to mandibular canal. In
maxilla- canine fossa
Periphery- Well-
defined, radiolucent rim;
sclerotic border
125

126. OSSIFYING FIBROMA
-Radiographic Features
Internal Structure-
Radio-opaque-radiolucent lesions
Sometimes— radiolucent
In the type that contains abnormal
trabeculae— pattern may be similar to
fibrous dysplasia
May be flocculent— cotton-ball appearance
Lesions with amorphous bone- solid,
homogenous radiopaque regions
Eversole- unilocular & multilocular
appearance
126

127. OSSIFYING FIBROMA
-Radiographic Features
Effect on Surrounding Structures-
Concentric growth- within medulla
Outward expansion in all directions-
cortical plate is thinned out but
INTACT
Teeth:-
Displacement- ✔
Root resorption- ✔
Lamina dura- Loss 127

128. OSSIFYING FIBROMA
-Radiographic Features
Effect on Surrounding Structures-
Mandibular canal:-
Displacement- ✔
Maxillary sinus:-
Can grow into & occupy most of the
sinus
Expands its wall outwards
Bony partition remains between them 128

129. OSSIFYING FIBROMA
-Differential Diagnosis
129
Ossifying fibroma Fibrous Dysplasia
Margins Better defined
Blends into surrounding
bone
Internal
structure
More variation
Less variation; more
homogenous in maxilla
Displacement
✔- displaces from an
epicentre
✔
Root resorption ✔ ✖
Expansion of
bone
✔- More concentric
about an epicentre
✔- Enlarges bone &
distorts shape— normal
morphology

130. OSSIFYING FIBROMA
-Differential Diagnosis
130
Ossifying fibroma PCOD
Single lesion Multifocal
Displacement ✔ ✖
Expansion ✔ Rarely
Pattern of
Expansion
Concentric Undulating
Ossifying fibroma Osteogenic Sarcoma
Thinning & INTACT Destroyed
Displacement ✖ ✔

131. OSSIFYING FIBROMA
-Histopathological Features
Fibrous stroma- highly
cellular
Hard-tissue portion— in
the form of osteoid or
cellular spherules
Variation in type of
mineralised material- not
seen in Fibrous dysplasia
131

132. OSSIFYING FIBROMA
-Management
132
Surgical enucleation/resection
Large lesions- several fragments
Recurrence- seldom seen except in younger
patients
Prognosis- excellent
No evidence of malignant transformation

133. JUVENILE OSSIFYING FIBROMA
133

134. JUVENILE OSSIFYING FIBROMA
-Introduction
Controversial lesion
Distinguished from the larger group of ossifying fibromas
based on
Age
Common site of involvement
Clinical behaviour
2 patterns have been noted- Trabecular & Psammomatoid
134

135. JUVENILE OSSIFYING FIBROMA
-Introduction
According to WHO,
“A fibro-osseous lesion that is characterised by
cellular rich fibrous tissue, bands of cellular
osteoid trabeculae and giant cells.”
135

136. JUVENILE OSSIFYING FIBROMA
-Clinical Features
AGE- Mean age are 11years and 22 years; 2-15
years
GENDER- No gender predilection (slight male
predilection)
SITE- Maxillary predominance
Psammomatoid- extra-gnathic sites ( 70%-
orbital, frontal bones & paranasal sinuses)
136

137. JUVENILE OSSIFYING FIBROMA
-Clinical Presentation
May be an incidental
finding
Sometimes- clinically
detectable facial
deformity
Pain & paraesthesia-
rarely
137

138. JUVENILE OSSIFYING FIBROMA
-Clinical Presentation
COMPLICATIONS- due to
impingement on
neighbouring structures
Nasal obstruction
Exophthalmos
Proptosis
Temporary/permanent
blindness
138

139. JUVENILE OSSIFYING FIBROMA
-Radiographic Features
Unilocular/multilocular
Location- In jaws > in maxilla.
Psammomatoid- in extra-gnathic sites
Periphery- Well-defined, radiolucent
rim; sclerotic border
Internal structure- Central
opacification
Effect on Surrounding Structures-
Cortical plate thinning & perforation
139

140. JUVENILE OSSIFYING FIBROMA
-Radiographic Features
CT findings- Well-defined
borders, thin sclerotic shell.
C o r t i c a l d i s r u p t i o n &
i n vo l v e m e n t o f a d j a c e n t
structures. More aggressive than
Fibrous dysplasia or ossifying
fibroma
MRI findings- Intermediate to
low signal intensity on MRI.
G r e a t e r s p e c i fi c i t y wh e n
n e u r o va s c u l a r o r o c u l a r
involvement is there.
140

141. JUVENILE OSSIFYING FIBROMA
-Histopathological Features
Not encapsulated
Well-demarcated from
surrounding bone
Cellular fibrous tissue- some
areas are highly cellular
whereas some may not be
Mitotic figures- are found
Mieralised component-
Trabecular & psammomatoid
141

142. JUVENILE OSSIFYING FIBROMA
-Management
142
Non-aggressive forms- conservative approach
Aggressive form- enbloc resection
Troulis & colleagues- 4 stages of treatment
Kaban & colleagues- 2 stages of treatment
( Aggressive JOF in maxilla & orbit)
Recurrence- 30-58%
No evidence of malignant transformation

143. A NOTE ON FEW OTHER SIMILAR APPEARING
LESIONS
143

144. NOTE ON FEW OTHER LESIONS
-Diagnostic Clues
144
CHERUBISM- Appears between 2-7years of age
Bilateral & symmetrical swelling of mandible
Radiographically- multilocular lesion in the mandible,
bilateral
Histopathologically- giant cells are preponderant
PAGET’S DISEASE- 3 PHASES; >40years of age; Max>> mandible
Bilateral presentation; radiolucent— cotton-wool appearance
Cortex-intact but thinned; linear horizontal trabecular pattern
Lumen of maxillary sinus is spared
HYpercementosis is seen loss of lamina dura; Resorption is
rare

145. NOTE ON FEW OTHER LESIONS
-Diagnostic Clues
145
CEMENTOBLASTOMA- 12-65years; relatively young; slight male predilection
Mandible>> maxilla— PM-M region
Well-defined with a radiolucent rim and a sclerotic border in the
surrounding bone
Mixed radilucent-radio-opaque- majority are radioopaque. Amorphous/
wheel-spoke pattern
If root outline is apparent— root resorption; mostly obscures the root
outline
May cause expansion with intact outer cortex
OSTEOBLASTOMA- Rare in the jaws, 5-22years
Most cases- condylar process; If in tooth-bearing areas— root resorption
Appears radiolucent solitary lesion

146. DIAGNOSING BASED ON VARIOUS
PARAMETERS
146

147. DIAGNOSING FEATURES
-Differential Diagnosis
147
BASED ON AGE
Before 30years After 30years
Cherubism Cemento ossifying fibroma
Fibrous dysplasia Cemento- osseous dysplasia
Juvenile ossifying fibroma Paget’s disease
BASED ON GENDER
FEMALES MALES
Cemento- osseous dysplasia Juvenile ossifying fibroma
Fibrous dysplasia

148. DIAGNOSING FEATURES
-Differential Diagnosis
148
BASED ON SITE OF OCCURRENCE
ANT. MANDIBLE ANT. MAXILLA POST. MANDIBLE POST. MAXILLA
PCOD
Juvenile OF
Ossifying fibroma
Ossifying
Fibroma
Fibrous
Dysplasia
Juvenile OF
FCOD
Florid COD
Gigantiform Cementoma

149. DIAGNOSING FEATURES
-Differential Diagnosis
149
BASED ON CLINICAL PRESENTATION
CLINICAL PRESENTATION Lesions associated
Swelling and facial
disfigurement
Fibrous dysplasia
Gigantiform cementoma
Incidental finding Cemento osseous dysplasia
Pain Juvenile Ossifying Fibroma
Self limiting Fibrous dysplasia
Continuous growth Neoplasms

150. FIBRO-OSSEOUS LESIONS
-Clinical Evaluation
150
Facial deformity
Vestibular obliteration
Overlying mucosa- same as the adjacent
tissues
Bony-hard in consistency
Non-tender on palpation
May show ulceration due to trauma
Displacement, malpositioning of teeth with
malocclusion
Mobility of teeth
H/o pain or paraesthesia— rarely

151. RADIOGRAPHIC DIAGNOSING FEATURES
-Differential Diagnosis
151
RADIOLUCENT STAGE- UNILOCULAR
TEETH NOT ASSOCIATED
Periapical granuloma Stafne bone cavity
Periapical cyst Osteoblastoma
Periapical abscess Cemento-ossifying fibroma
PCOD
Cemento-ossifying fibroma

152. RADIOGRAPHIC DIAGNOSING FEATURES
-Differential Diagnosis
152
RADIOLUCENT STAGE- MULTILOCULAR
TEETH NOT ASSOCIATED
Odontogenic myxoma Central Giant Cell Granuloma
Glandular Odontogenic Cyst Juvenile Ossifying Fibroma
Ameloblastoma Hyperparathyroidism
Juvenile Ossifying Fibroma Metastatic tumours of the jaws
Ossifying Fibroma Ossifying Fibroma
Cherubism

153. RADIOGRAPHIC DIAGNOSING FEATURES
-Differential Diagnosis
153
MIXED RADIOLUCENT-RADIOPAQUE STAGE
TEETH NOT ASSOCIATED
PCOD FCOD
Ossifying Fibroma Florid Cemento-osseous Dysplasia
Fibrous Dysplasia
Paget’s Disease
Ossifying Fibroma
Osteogenic Sarcoma
Desmoplastic Ameloblastoma

154. RADIOGRAPHIC DIAGNOSING FEATURES
-Differential Diagnosis
154
RADIO-OPAQUE STAGE
TEETH GENERALISED
Condensing osteitis Florid Cemento-osseous Dysplasia
PCOD Paget’s Disease
FCOD Familial Gigantiform Cementoma
Cemento-ossifying fibroma Fibrous Dysplasia
Cementoblastoma Osteopetrosis
Complex odontoma
Hypercementosis

155. RADIOGRAPHIC DIAGNOSING FEATURES
-Differential Diagnosis
155
CHARACTERISTIC RADIOGRAPHIC APPEARANCES
Radiographic appearance Lesions associated
Cotton-Wool
1. Florid Cemento-osseous Dysplasia
2. Paget’s Disease
3. Ossifying Fibroma
4. Fibrous Dysplasia (adult monostotic)
Ground-glass
Fibrous Dysplasia
Paget’s disease
Orange-peel Fibrous Dysplasia
Chalky type
Osteopetrosis
Fibrous dysplasia
Wheel-spoke pattern Cementoblastoma

156. FIBRO-OSSEOUS LESIONS
-Conclusions
156
Thus, a working knowledge of the clinical
features, presentation and radiographic features
is of utmost importance in identifying and
diagnosing a fibro-osseous lesion to help in
providing guidance in chosing the right treatment
modality for the patient.

157. Thank you…