This is a presentation of Fibro-Osseous lesions of head & neck region from the point of view of an Maxillo-Facial Surgeon

1. FIBRO-OSSEOUS LESIONS
Guided by: Dr. M E SHAM
Presented by: Dr. RAYAN

2. CONTENTS
• INTRODUCTION
• CLASSIFICATION OF FIBRO-OSSEOUS LESIONS OF THE JAWS
• FIBROUS DYSPLASIA
• CEMENTO-OSSEOUS DYSPLASIA
• FOCAL CEMENTO-OSSEOUS DYSPLASIA
• PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA
• FLORID CEMENTO-OSSEOUS DYSPLASIA
• OSSIFYING FIBROMA
• JUVENILE AGGRESSIVE OSSIFYING FIBROMA
• CHERUBISM
• CONCLUSION
• REFERENCES

3. • 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.
• A varied group of lesions characterized by
replacement of normal bone by tissue composed
of collagen and fibroblasts, with variable amounts
of a mineralized substance that may be bone,
cementum or both.
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

4. • Waldron described fibro osseous lesions as a group of
pathological changes within 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. J Oral Maxillofac Surg. 1985
Apr;43(4):249-62.
DEFINATIONS

5. Classifications

6. • Charles Waldron Classification Of The Fibro-Osseous Lesions Of
The Jaws (1985)
• Classification Of Fibro-Osseous Lesions By Mico M. Malek
(1987)
• Peiter J. Slootweg & Hellmuth Muller (1990)
• WHO Classification (1992)
• Waldron Modified Classification Of Fibro-Osseous Lesions Of
Jaws (1993)
• Brannon & Fowler Classification (2001)
• WHO Classification Of Fibro-Osseous Lesions Of Jaws (2005)
• Paul M. Speight & Roman Carlos Classification (2006)
• Eversole Classification (2008)
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

7. Waldron (1985):
I. Fibrous dysplasia (FD)
A. Polyostotic
B. Monostotic
II. Fibro-osseous (cemental) lesions presumably arising in the periodontal
ligament
A. Periapical cemental dysplasia
B. Localized fibro-osseous-cemental lesion (probably reactive in nature)
C. Florid cement-osseous dysplasia (gigantiform cementoma)
D. Ossifying and cementifying fibroma
III. Fibro-osseous neoplasms of uncertain or debatable relationship to those
arising in the periodontal ligament
A. Cementoblastoma, osteoblastoma and osteoid osteoma
B. Juvenile active ossifying fibroma and other so-called aggressive, active
ossifying/cementifying fibromas.

8. Classification Of Fibro-Osseous Lesions By Mico M. Malek
(1987)
1. Developmental Disorders
A. Fibrous Cortical Defects
B. Fibrous Dysplasia
2. Reactive Reparative Lesions
A. Traumatic Periostitis
B. Periostitis Ossificans
C. Osseous Keloid
D. Periapical Cemental Dysplasia & Florid Cemento-
Osseous Dysplasia
E. Sclerosing Osteomyelitis (Focal & Diffuse Type)
F. Osteitis Deformans

9. 3. Fibromatosis
A. Desmoplastic Fibroma (Intraosseous Fibromatosis)
4. Neoplasms
A. Tooth Bearing Areas Only
i. Cementoblastoma
ii. Periodontoma
1. Central
2. Peripheral
B. All Cranio-Facial Bones (Including Tooth Bearing Areas)
i. Osteoma
1. Trabecular
2. Compact
ii. Osteoid Osteoma
iii. Psammous Desmo-Osteoblastoma
iv. Trabecular Desmo-Osteoblastoma

10. Peiter J. Slootweg & Hellmuth Muller
(1990)
• Group I: Fibrous Dysplasia
• Group II: Juvenile Ossifying Fibroma
• Group III: Ossifying Fibroma
• Group IV: Periapical Cemental Dysplasia & Florid
Osseous Dysplasia

11. WHO Classification (1992)
1. Osteogenic Neoplasms
a. A.Cemento-Ossifying Fibroma (Cementifying Fibroma, Ossifying
Fibroma)
2. Non-Neoplastic Bone Lesions
a. Fibrous Dysplasia Of Jaws
b. Cemento-Osseous Dysplasia
I. Periapical Cemental Dysplasia (Periapical Fiberous Dysplasia),
II. Florid Cemento-Osseous Dysplasia (Gigantiform
Cementoma, Familial Multiple Cementomas)
III. Other Cemento-Osseous Dysplasia
c. Cherubism (Familial Multilocular Cystic Disease Of The Jaws)
d. Central Giant Cell Granuloma
e. Aneurysmal Bone Cyst
f. Solitary Bone Cyst (Traumatic, Simple, Hemorrhagic Bone Cyst)

12. Waldron Modified Classification Of
Fibro-Osseous Lesions Of Jaws (1993)
1. Fibrous Dysplasia
2. Cement-Osseous Dysplasia
a. Periapical Cement-Osseous Dysplasia
b. Focal Cement-Osseous Dysplasia
c. Florid Cement-Osseous Dysplasia
3. Fibro-Osseous Neoplasm
a. Cementifying Fibroma, Ossifying Fibroma, Cement Ossifying
Fibroma

13. Brannon & Fowler Classification (2001)
1. Osseous Dysplasia (OD) (Reactive)
a. Nonhereditary
i. Periapical
ii. Focal
iii. Florid
b. Hereditary (Developmental)
i. Familial Gigantiform Cementoma
2. Fibro-Osseous Neoplasm
a. Ossifying Fibroma (OF)
b. “Juvenile”, “Active” or “Aggresive” Varients of OF

14. 3. Fibrous Dysplasia
a. Polyostotic FD
b. Monostotic FD
c. Craniofacial FD
4. Giant Cell Lesions
a. Central Giant Cell Granuloma
b. Aneurismal Bone Cyst
c. Cherubism
5. Miscellaneous Benign Fibro-Osseous Lesions
a. Cementoblastoma
b. Tori/Exostoses
c. Osteoma

15. 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
4) Central Giant Cell Granuloma
5) Cherubism
6) Aneurismal Bone Cyst
7) Solitary Bone Cyst

16. Paul M. Speight & Roman Carlos Classification (2006)
1. Fibrous Dysplasia
a. Monostotic FD
b. Polyostotic FD
c. Craniofacial FD
2. Osseous Dysplasia
a. Periapical Osseous Dysplasia
b. Focal Osseous Dysplasia
c. Florid Osseous Dysplasia
d. Familial Gigantiform Cementoma
3. Ossifying Fibroma
a. Conventional Ossifying Fibroma
b. Juvenile Trabecular Ossifying Fibroma
c. Juvenile Psammomatoid Ossifying Fibroma

17. Eversole 2008 Classification

18. Radiographs of clinical cases were classified into 5 patterns. (a) Focal pattern,
radiopacity with uniformity ; (b) target pattern, central radiopacity within radiolucent
area; (c) lucent pattern, well-defined radiolucent area ; (d) calcification pattern,
calcified flecks or scattered within radiolucent area ; (e) multiconfluent pattern,
radiopacity with mottled pattern

19. FIBROUS DYSPLASIA

20. • Initially described as “osteitis fibrosa generalisata” by von
Recklinghausen in 1891
• Lichtenstein in 1938 coined this term.
• Also called as-
Fibrocystic disease,
Osteitis fibrosa localisata,
Focal osteitis fibrosa,
Fibroosteodystrophy.
•They constitute 7% of all non malignant bone tumors.
Ricalde P, Horswell BB. Craniofacial fi brous dysplasia of the fronto-orbital region: A case
series and literature review. J Oral Maxillofac Surgery 2001;59:157-67.

21. 21

22. • It is a non-heritable, non-malignant condition in which normal
bone and marrow is replaced by fibrous tissue and
haphazardly distributed woven bone.
• 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

23. • 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

24. Etiopathogenesis
• Many theories which have tried to explain the genesis of fibrous
dysplasia.
1. Excessive production of interleukin-6 at local sites has been related
to increased resorption of bone by increasing the numbers of
osteoclasts in these lesions.
2. Genetic theory postulates that there somatic mutation early in
embryonic life.
- The earlier the mutation occurs, the more widespread the effects
will be.
- The gene is located on band 20q13.2, an area that codes for the α
subunit on G-protein receptors Mutations in the gene (GNAS I) result
in a cascade which may lead to alteration in cellular differentiation
and osteoblastic proliferation.

25. 3. Hormonal theory-
- osteoblasts in fibrous dysplastic lesions have an elevated number of
hormone receptors and thus have altered responses of bone
formation.
- Hormonal alteration occurring during life, like in pregnancy usually
sees exuberated growth of these lesions, thus explaining its hormonal
genesis.
4. Another theory explains that cAMP also activates Fos, which inhibits
osteoblastic specific genes as well as stimulating cytokines that
promote bone resorption by osteoclasts.
• Hypophosphatemia/phosphaturia, sometimes found in FD, is caused
by excess secretion of a phosphatonin fibroblast growth factor

26. Classification
2 types
1. Monostotic
2. Polyostotic -
a. Jaffe type
b. Albright’s syndrome

27. Clinical Features
1. The solitary (monostotic) form of fibrous dysplasia-
Only one bone is involved.
Accounts for 70% of all cases.
Is the type that most often involves the jaws.
The most common sites (in order) are the ribs, femur, tibia, maxilla
and mandible.
Is discovered in a slightly older age group.

28. 2. The multiple (polyostotic) form –
• More than one bone is involved.
• Usually found in children < 10 yrs.
• Accounts for 20-25% of all FDs.
a. Jaffe type-
Involving variable no. of bones accompanied by pigmented lesions of
skin or café au lait spots
b. Albright’s syndrome-
A severe form involving nearly all the bones of the
body accompanied by pigmented lesions of skin &
endocrine disturbances

29. CAFÉ-AU-LAIT
French word for
Coffee with milk

31. • The monostotic form is more commonly found in the facial skeletal
region.
• Involves the maxilla almost twice as often as the mandible and
• Occurs more frequently in the posterior aspect.
• Commonly they are unilateral except for very rare extensive lesions
of the maxillofacial region that are bilateral.
• Patients may first complain of unilateral facial swelling or an
enlarging deformity of the alveolar process.
• If extensive craniofacial lesions have impinged on nerve foramina,
neurologic symptoms may develop.

32. • The lesions usually become static when skeletal growth stops,
• but proliferation may continue, particularly in the polyostotic
form.
• It causes painless expansile lesions that impair cosmetic and
structural function of bone.

34. • Leontiasis Ossea means Lion-Face.
• This is an appearance due to overgrowth of cranio-facial
bones.
• More often it affects the maxilla.

35. • The rates of malignant transformation to
osteosarcoma have been estimated to be about
0.5% with monostotic FD and about 4% with
McCune-Albright syndrome.
Jhala DN, Eltoum I, Carroll AJ, et al. Osteosarcoma in a patient with McCune-Albright
syndrome and Mazabraud’s syndrome: a case report emphasizing the cytological and
cytogenetic findings. Hum Pathol. 2003; 34(12):1354–1357.

36. Radiographical features -
• 6 types:
1. The pagetoid type – characterized by bone expansion and alternate
areas of radiodensity and radiolucency as seen in Paget’s disease .
2. The sclerotic type- Appears as bone expansion with homogeneous
radiodensity, giving the classic “ground-glass appearance”.
The normal bone is replaced by tissue that is more radiolucent, with a
grayish "ground-glass" pattern that is similar to the density of
cancellous bone but is homogeneous, with no visible trabecular
pattern.
3. The cyst like type- appears as solitary, round or oval well defined
radiolucent lesion with sclerotic margin.

37. 4. Peau d’ orange or orange peel type –
Alternating areas of granular density & lucency giving a radiographic
appearance resembling the ring of orange
5. Whorled plaque like type-
The matrix of well circumscribed lesion is composed of plaques of
amorphous material of intermediate radiodensity which are seen to
be arranged in whorled onion peel appearance
6. Chalky type- is a well circumscribed lesion consisting of an
amorphous dense radiopaque material

39. Intraoral x rays –
‘Orange peel’
appearance
Extra-oral radiographs –
‘Ground glass’ (frosted glass)
appearance
39

40. Histological Features
• Proliferation fibroblasts in compact stroma of interlacing
collagen fibres
• The appearance of numerous short, irregularly shaped trabeculae of
woven bone.
• These trabeculae are not aligned in response to stress but rather
have a random orientation.
• Some of trabeculae are c – shaped & described as chinese character
shaped.
• This histologic appearance is responsible for the internal pattern
seen in radiographs.

41. CHINESE LETTERS

44. LAB STUDIES
• Serum alkaline phosphatase levels are often elevated depending on
the extent of bony disease.
• Serum calcium, phosphate, and vitamin D levels are assessed to assist
in excluding rickets.
• Thyroid function tests - to exclude hyperthyroidism.

45. Treatment options
• Recommended treatment options can be divided into 4
categories:
1. Observation
2. Medical therapy
3. Surgical remodelling
4. Radical excision and reconstruction

46. • In 1990 Chen and Noordhoff proposed a treatment algorithm for the
management of craniomaxillofacial fibrous dysplasia incorporating
aggressive, radical surgery for the resection of diseased tissue.
• They proposed that the head and face could be divided into 4 zones
based on the esthetic and functional consequences of the disease
Chen YR, Noordhoff MS: Treatment of craniomaxillofacial fibrous
dysplasia: How early and how extensive? Plast Reconstr Surg 86:835, 1990

47. • 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.
- For this region, they recommended
radical excision and reconstruction.
• Zone 2
- Refers to the hair bearing scalp.
- It is not typically an aesthetic
concern, and as such, intervention is
optional for the patient.

48. • 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 recommended conservative
management, given the difficulty in
reconstructing defects in this region.

49. Treatment and Prognosis:
• There is no spontaneous resolution of FD.
• Not all patients require treatment.
• Surgery may be indicated if bone deformity is large and causes
compression of adjacent tissue.
• Bisphosphonates are often used as medical treatment as they may
reduce the increased bone resorption
• Some patients with minimal cosmetic or functional deformity may
not require or desire surgical treatment.
• Cosmetic deformity with associated psychologic problems or
functional deformity may dictate surgical intervention in the younger
patient.

50. • The prevalence of re growth after surgical reduction is difficult to
determine
• 25% and 50% of patients show some re growth after surgical shave-
down of the lesion.
• The re growth is more common in younger patients,
• Many surgeons believe that surgical intervention should be delayed
for as long as possible.

51. • Third generation bisphosphonates (zolendronic acid) have shown
remarkable success rate in relieving bone pain and healing of lesions.
• Intravenous 4 mg zolendronic acid along with vitamin D and oral
calcium supplements is the choice of treatment

52. • some authors have advocated aggressive surgical resection –
- When rapid enlargement occurs,
- adjacent vital structures, such as the optic nerve, globe and auditory
canal/ structures and nasal airway may be invaded or compressed,
resulting in functional deficits.
- to avoid potential blindness or hearing loss.
- Rapid enlargement of FD in the nasal bones, maxilla or mandibular
symphysis may result in airway obstruction by obliteration of the
nasal cavity or by posterior displacement of the tongue.

53. • In patients with non-aggressive but active FD
- ideal is to wait until the lesion becomes quiescent and
- the patient has reached skeletal maturity before performing an
operation.
• In cases of PFD where the disease is extensive, the lesions are often
not resectable
• Repeat surgical contouring and extensive debulking may be necessary
to achieve acceptable facial proportions.

54. • In the future improvement in CT imaging and software will allow for
accurate surgical simulation and intraoperative navigational tools may
guide the surgeon throughout the contouring.
• Advanced CT software is useful for superimposition of pre- and
postoperative images.
• These can then be compared to follow up CT scans to determine
stability of the result or the presence of regrowth.
• Despite these new imaging technologies, there is no therapy or
technology that can predict and/or prevent regrowth.

55. • The etiology of change in behavior causes include:
- associated expansile lesions such as , malignant transformation,
and osteomyelitis.
• A biopsy of the area of growth is necessary prior to surgical
management.
• Treatment may range from contour resection to en bloc resection
depending on the diagnosis.
• In cases of an associated lesion, the management based on that
associated lesion.

56. SURGICAL THERAPY
• Surgical indications can be summarized as follows:
– Rapidly progressive or aggressive disease
– Impending orbital encroachment
– Gross facial asymmetry or disfigurement
– Sudden enlargement with onset of pain – malignant degeneration
– Other functional or cosmetic disability
56

57. Nonsurgical and adjuvant management of craniofacial FD
• While pain is common among FD patients
• Kelly et al examined
- 78 patients (35 children and 43 adults) and
- found 67% complained of pain.
- some patients required NSAIDs with and without narcotic treatment,
• In the same study –
- approximately 20% of the patients were managed with bisphosphonates and
- nearly 75% reported pain relief or improvement with this class of drugs.

58. • Malignant transformation is most often osteosarcoma but
fibrosarcoma, chondrosarcoma, and malignant fibrohistiocytoma
have also been reported
• Radiation therapy for FD is contraindicated because it carries the risk
for development of post irradiation bone sarcoma.

59. 59
• Conclusion:
– If possible postpone surgery till lesion growth subsides
– Conservative shaving or osseous contouring gives desired results
– Periodic contouring in cases with continued growth – less total
morbidity than single massive resection
– In indicated cases of radical excision – reconstruction with
replantation of resected bone after autoclaving
• Maintains original anatomy
• Reliable
• Eliminates donor site morbidity
• Provides good immediate clinical result
• No recurrence

60. • 1st described by Robinson in 1956
• Introduced term – “Osseous dysplasia” & attributed the cause as
reaction of bone to local injury.
• Origin – Periodontal ligament / extra-ligamentary bone
• Triggered by local factors / underlying hormonal imbalance
• Types
Non hereditary
– Periapical
– Focal
– Florid
Hereditary
– Familial Gigantiform cementoma
Cemento-osseous Dysplasia
60

61. • 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.
Miloro M, Ghali GE, Larsen P, Waite P. Peterson's principles of oral and maxillofacial
surgery; 3rd edition; volume 1; PMPH-USA; 2004.

62. 62

63. (a) Periapical osseous dysplasia
• Osseous dysplasia;
• Cemental dysplasia;
• Periapical cementoma;
• Periapical FD;
• Periapical ossifying fibroma
• Is a reactive fibro-osseous lesion
• Thought to arise from elements in the versatile periodontal ligament,
• Predilection for black females and Develops almost exclusively after
the age of 30 years.
• Almost always asymptomatic and detected during a routine
radiographic examination.
• Is usually found in intimate association with the root apices of the
mandibular anterior teeth

64. • classically described as progressing through 3 radiographic stages.
1) Osteoloytic stage
2) Cementoblastic stage.
3) The third or mature stage

65. • The early area shows –
- proliferating fibrous connective tissue with no evidence of an
inflammatory infiltrate.
- Small foci of cementum, osteoid or bone are almost
invariably present.
• Advanced lesions show
- a greater proportion of mineralized, cementum like material or
- thick, sclerotic bone trabeculae or an admixture of both.

66. (b) Focal osseous dysplasia
• presents as a solitary lesion in the posterior jaws, most often the mandible.
• Waldron suggested that focal osseous dysplasia likely represents the most
common benign fibro-osseous lesion of the jawbones.
• This condition is invariably asymptomatic,
• manifesting most commonly as a small, solitary, relatively well-demarcated
lesion in the posterior mandible, either in close association with the apices
of teeth or in areas where a tooth has been extracted previously.
• is much more common in black females than males.
• most lesions are recognized during the fourth and fifth decades of life.
• Radiographically focal osseous dysplasia tends to manifest as an irregularly
shaped, mixed radiolucent-radiopaque lesion, occasionally with well-
defined borders.

68. 68
Focal Cemento-osseous Dysplasia

69. (c) Florid osseous dysplasia
• florid cemento-osseous dysplasia;
• gigantiform cementoma;
• familial multiple cementomas
• termed florid because of their widespread, extensive manifestation.
• normal cancellous bone is replaced with dense, acellular cemento-
osseous tissue in a background of fibrous connective tissue.
• However, if periapical cemental dysplasia is defined in three or four
quadrants or is extensive throughout one jaw, it usually is considered
to be florid osseous dysplasia.

70. • predominantly involves black women with a marked
predilection for middle-aged to the elderly.
• The lesions show a marked tendency for bilateral and
often quite symmetric involvement,
• it is not unusual to encounter extensive lesions in all
four posterior quadrants.
• Both dentulous and edentulous areas may be affected.

72. 72
Florid Cemento-osseous Dysplasia

73. • All three patterns of cemento-osseous dysplasia demonstrate
similar histopathologic features.
• The tissue consists of fragments of cellular mesenchymal tissue
composed of spindle shaped fibroblasts and collagen fibers with
numerous small blood vessels.
• Within this fibrous connective tissue background dense,
sclerotic masses which have been interpreted as cementum is
seen.
• As the lesions mature and become more sclerotic, the ratio of
fibrous connective tissue to mineralized material decreases.

74. Treatment and Prognosis
• The various forms of cemento-osseous dysplasia do not appear
neoplastic; therefore, they generally do not require removal.
• For the asymptomatic patient, the best management consists of
regular recall examinations with prophylaxis and reinforcement of
good home hygiene care to control periodontal disease and prevent
tooth loss.
• Because the onset of symptoms is usually associated with exposure
of the sclerotic masses to the oral cavity, biopsy or elective extraction
of teeth should be avoided.
• In other instances, symptoms begin after exposure of the sclerotic
masses to the oral cavity as a result of progressive alveolar atrophy
under a denture.

75. • Sequestration of the sclerotic cementum-like masses occurs
slowly and is followed by healing.
• Saucerization of dead bone may speed healing.
• When simple bone cysts arise within foci of cemento-osseous
dysplasia –
- surgical exploration is necessary to establish the
diagnosis.
- To assist healing, the cyst and the surrounding fibro-
osseous proliferation are usually curetted thoroughly.

76. III. Ossifying fibroma
• cementifying fibroma;
• cementoossifying fibroma
• Montgomery was first to coin the term “ossifying fibroma tissue within which the
bone is formed.
• accounts for only 0.1% of the bony lesions.
• belongs to the poorly defined group of fibroosseous lesions involving the jaws and
craniofacial bones that results in replacement of the bone by fibrous tissue and
subsequent mineralization.
• The cause remains unknown.
• usually occurs between the 3rd and 4th decade of life with the average age being
30 yrs.
• Marked predilection in females with female to male ratio varying from 1.56:1 to
5:1.

77. 77

79. • Unlike FD, ossifying fibromas are characteristically monostotic.
• Less than 5% involve more than one bone and they are almost
exclusively found in the cranial bones,
• Of the cranial bones, the mandible is the most common site (75% in
some series),
followed by rarer reports of the ethmoid, frontal, and sphenoid
sinuses, as well as the orbit, occiput, and temporal bone.

80. • Goaz and White reported that when OF occurs in the maxilla, it is
most commonly located in the canine fossa and zygomatic arch.
• It may grow to completely fill the maxillary sinus.
• It can effect both maxilla and mandible
• mandible varying from 70%-89% of cases and maxilla in 11%- 26%
• with affinity for premolar & molar area.
• The maxillary lesions were found to be more aggressive.

81. • They are associated with a slowly progressing enlargement of the
affected bone.
• Lesion is asymptomatic until the growth produces a notable
swelling and mild deformity and facial asymmetry.
• Displacement of teeth is an early clinical feature.
• When rapid growth does occur, the symptoms are related to the
lesion site and may include painless cheek swelling, unilateral
proptosis, diplopia and epistaxis.
• Death is a rare occurrence secondary to intracranial extension.

82. • Clinical features
– Displacement of teeth – first clinical
symptom
– Nasal obstruction, headache, bone
pain, and proptosis – reported in
cases with periorbital &
juxtasinonasal lesions
– Overlying cortical bone and mucosa –
intact
– Encroachment on contiguous
structures.
– In the orbit they may displace the
globe
82

83. • These lesions may occasionally have ill-defined border, if relatively
rapid growth occurs.
• As the lesion matures, mixed radiolucent and radiopaque appearance
may be seen.
• The characteristic features are expansion and lesion margination,
demarcation, or cortication.
• Cortical expansion is present, often with an eggshell- thin cortex.
• Large ossifying fibromas of mandible often demonstrate a
characteristic downward bowing of inferior cortex of mandible.

84. Radiological features
 Characteristic features : expansion and lesion margination,
demarcation, or cortication
84
Ossifying Fibroma

85.  Well defined unilocular radiolucency – sclerotic border,
delineated from adjacent
bone by a radiolucent halo
 Root resorption, root
divergence
85

86. • Early stage : Radiolucent,
may / may not contain
floccular opacities.
• Late Stage : mineralized
tumors, many radiopaque
and confluent foci
86

87. • Gross
– Relatively hypovascular
– Well demarcated – from surrounding tissue
– Centrifugal growth pattern – maintains round/oval
shape with enlargement
– Encapsulated
– Easy separation
87

88. • the tumor is relatively hypovascular and well demarcated, permitting
relatively easy separation from the surrounding bone.
• Some lesions will have a definite capsule.
• This demarcation from the surrounding tissue is an important feature
in distinguishing OF from FD.
• The calcified component consists of rounded or lobulated basophilic
cementum-like masses, trabeculae of osteoid or bone or
combinations of both,
• the majority of bony trabeculae in cementoossifying fibroma are
thin, single, and separate with osteoblastic rimming.

89. • Histopathologic features
– Rimmed woven bone replaced by layers of lamellar
bone
– Fibrous tissue – varying degrees of cellularity
– Mineralized material
• Trabeculae of woven and lamellar bone
• Basophilic poorly cellular spherules
– May be areas of cementum appearing as
Psammoma bodies embedded in benign fibrous stroma
89

90. Bony trabeculae
• Vary in size
• Mixture of woven and lamellar
pattern
• Osteoblastic rimming
Droplets of cementum like matter –
Psammoma bodies
Ossifying Fibroma

91. • Treatment and Prognosis
• The circumscribed nature of the ossifying fibroma generally
permits enucleation of the tumor with relative ease. Some
examples, however, which have grown large and destroyed
considerable bone, may necessitate surgical resection and bone
grafting. The prognosis, however, is very good, and recurrence
after removal of the tumor is rarely encountered. There is no
evidence that ossifying fibromas ever undergo malignant
change.

92. Juvenile ossifying fibroma
• Juvenile (aggressive) ossifying fibroma was used in 2nd
edition of WHO classification of odontogenic tumor of
children to describe a lesion affecting the jaws under the age
of 15 years.
• Definition
• The second edition of the WHO classification of odontogenic
tumors defines juvenile (aggressive) ossifying fibroma as an
actively growing lesion consisting of cell rich fibrous tissue
containing bands of cellular osteoid without osteoblastic
rimming together with trabeculae of more typical bone.
• Giant cells may also be present.

93. • Classification
• It is the term used to describe two distinct histopathologic
variants of ossifying fibroma of the craniofacial skeleton –
• psammomatoid juvenile ossifying fibroma
• trabecular juvenile ossifying fibroma

94. • The clinical signs and symptoms are related to the anatomic
site of involvement.
• The tumor probably originates in early childhood, but
enlarges slowly, resulting in its delayed detection in adults. In
most patients (85%), the tumors are located in the facial
bones, but they also involve the calvaria (12%) and
extracranial sites (4%).
• Among facial lesions, 90% arise from paranasal sinuses and
the remaining 10% arise from the mandible, perhaps from
maldevelopment of the tissue generating the bony septa
between the roots of molar teeth.

95. • The tumor may manifest as well-demarcated, unilocular or
multilocular radiolucencies with a variable amount of
radiopacity, usually manifesting as fine specks or as scattered,
irregularly shaped bony trabeculae and calcified spherules
amid a background of relatively avascular, cellular fibrous
tissue.

96. Treatment and Prognosis:
• The clinical management and prognosis of the juvenile
ossifying fibroma are uncertain. Although many tumors
demonstrate slow but progressive growth, some
juvenile ossifying fibromas demonstrate rapid
enlargement. The more aggressive neoplasms tend to
arise in infants and young children.
• For smaller lesions, complete local excision or thorough
curettage appears adequate. For some rapidly growing
lesions, wider resection may be required.
• In contrast to the negligible recurrence rate seen in the
common types of ossifying fibromas, recurrence rates of
30% to 58% have been reported for juvenile ossifying
fibromas. Malignant transformation has not been
documented.

97. CHERUBISM
CHERUB – ANGEL

98. • familial intraosseous fibrous expansion of the mandible,
• is a genetically-mediated disorder
• first reported in 1933.
• The word ‘Cherubism’ refers to the spherical facial appearance of angels
painted in the Renaissance era.
• is a non-neoplastic hereditary bone lesion
• Characterized by a spherical and symmetrical chubby facial appearance,
bilateral painless swelling of the maxilla and mandible, resulting in a fullness
of the cheeks and retraction of the lower eyelids, giving an upward turned
appearance of the eyes – comparable to a cherub angel.

99. • It is an autosomal dominant inheritance in 80% of familial
patterns.
• Typically, the mandible is primarily affected and, in 60% of
cases, maxilla is also involved.
• Children are normal at birth.
• At the age of 14 months to 5 years, a symmetric enlargement
of the jaws begins

100. • males at a proportion of 2 : 1,
• enlarged face due to swelling of the jaws which is bilateral in most cases,
• bone consistency of the lesion,
• intact mucosa,
• dental malocclusion,
• upward-looking eyes in the case of maxillary involvement, and
• absence of pain.
• The concomitant presence of cervical and/or submandibular lymphadenopathy has
been reported.
• The first signs of manifestation of the disease are generally observed at about 2
years of age, followed by accelerated growth from 8 to 9 years and spontaneous
interruption after puberty.

101. Dental alterations
• early exfoliation of deciduous teeth,
• impaction and/or displacement of teeth, which radiographically seem
to float in radiolucent areas, conferring the so-called “floating tooth
appearance”.
• In addition, ectopic tooth eruption, agenesis of permanent teeth,
mainly of the second and third molars, due to involution of their
germs
• root resorption of existing teeth are observed.
• These alterations result in malocclusion as well as in problems of
phonation and swallowing

102. Biochemical parameters
• serum calcium and phosphorus concentrations and TSH, FSH, LH, T4
and T3 hormone levels are usually within normal limits,
• but alkaline phosphatase levels might be elevated.
• A constant finding in these patients is the enlargement of
submandibular and cervical lymph nodes.

103. Radiographic features
• lesions appear as cystic multilocular radiolucencies clearly bounded by cortical
bone and distributed bilaterally in the posterior quadrants of the mandible and/or
maxilla,
• often beginning near the angle of the mandible and spreading to the mandibular
ramus and body.
• Maxillary lesions may concomitantly occur, usually in the maxillary tuberosity
region.
• Frequently, teeth appear as displaced and impacted,
• root resorption is observed;
• frequently, the mandibular canal is also displaced.

104. Radiographic grading of Cherubism
• Arnott suggested radiographic staging for the lesions of Cherubism according to
their location and degree of expansion.
• Ramon and Engelberg modified grading based on the area of involvement, as
follows:
• Grade I: involvement of both mandibular ascending rami.
• Grade II: involvement of both maxillary tuberosities, as well as of the mandibular
ascending rami.
• Grade III: massive involvement of the whole maxilla and mandible, except the
coronoid process and the condyles, resulting in considerable facial deformity.
• Grade IV: grade 3 plus the involvement of the floor of the orbits, causing orbital
compression.
• The grade may change depending on findings at follow-up examination.

105. Histological subtypes
(Peñarrocha et al. Cherubism. J Oral Maxillofac Surg 2006.)
• Subtype I: Great abundance of large giant cells totally occupying the lesion surface.
High cell density: 160 to 180 giant cells per _200 field, intermingling with small
areas of interstitial microhemorrhage.
• Subtype II: Lesser presence of giant cells, which represent only part of the lesion.
Abundant active inflammatory component, with extensive vascularization,
associated to giant cells of variable size. Low cell density: 10 to 15 giant cells per _
200 field, intermingling with inflammatory and interstitial microhemorrhagic areas.
• Subtype III: Abundant fibrosis with a reduced presence of giant cells. The
inflammatory component is scarce, although there may also be interstitial
microhemorrhagic areas. Low cell density: 5 to 8 giant cells per _200 field,
associated to an extensive and often dense or highly cellular fibrotic component.

106. Treatment
• generally a self-limiting lesion, which spontaneously regresses with
age.
• Jaw remodelling continues through the third decade of life, at the
end of which the clinical abnormality may be subtle.
• The frequency of remodeling is unknown, since most of the recorded
cases have been surgically treated before reaching puberty.
• Conventional treatment of cherubism includes jaw contouring,
curettage of the lesions, and management of dental disharmony.
• Curettage alone or in combination with surgical contouring has been
considered the treatment of choice,

107. • Liposuction has been used to change the contour of the jaws in
patients with cherubism.
• Some authors point medical therapy in the form of calcitonin, as a
possibility to curtail the disease and obviate the need for surgery.
• Calcitionin has been shown to cause inhibition of bone resorption
by multinucleate cells in cherubic tissue in vitro.
• The daily use of 200 IU salmon calcitonin via nasal spraying for
cherubism has been recently reported.

108. • Based on the genetic mutations related to the disease, gene therapy is expected to
play a role in future treatments.
• Treatment options include –
- waiting for stabilization and spontaneous remission of the disease,
- tooth extraction in areas showing fibrous alterations,
- cosmetic osteoplasty of the affected jaws after regression of disease
activity or, in functional impairment,
- curettage of the lesions and
-treatment with calcitonin.
• The policy of waiting for disease regression, followed by the evaluation of
physiological bone remodelling, is the most recommended.
• Curettage has been suggested to be as a good approach since this intervention
stimulates bone replacement.

109. • According to Novack and Faccio hypothesis that cherubism is caused
by enhanced cytokine tumour necrosis factor α (TNF-α) production
by myeloid cells due to an activating mutation in Sh3bp2 not only
represents a major advancement in the understanding of the disease
but suggests new potential options for its treatment.
• Anti-TNF therapies are already in clinical practice.

110. • FOL exhibit a variety of clinical behavior, but share
microscopic features of a benign connective tissue
matrix & new bone formation
• Overlapping histologic features & atypical features of
individual lesions make definitive diagnosis difficult
• Based on history, clinical, histological & radiographic
features, a definitive diagnosis should be actively
sought so that the lesions are not treated incorrectly
11
0
Conclusion

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