Fibrous dysplasia

1. FIBROUS DYSPLASIA
SUPERVISED BY PROF DR AYMAN MADNY
PREPARED BY
EMAN ZAYED

2. CONTENTS

 Introduction
 Classification
 Etiology
 Clinical Features
 Monostotic Form
 Polyostotic Form
 Craniofacial form
 Radiographic Features
 Histologic Features
 Treatment &Prognosis
 Conclusion

3. Fibro-Osseous
Lesion
• “Replacement of normal bone by tissue composed of
collagen fibers and fibroblasts, containing varying
amount of mineralized substance” – Waldron 1985.
• “A collection of non – neoplastic intraosseous lesions
that replace bone and consists of a cellular fibrous
connective tissue within which non- functional osseous
structures form” Eversole
INTRODUCTION


4. 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. Aneurismal Bone Cyst
f. Solitary Bone Cyst (Traumatic, Simple, Hemorrhagic Bone Cyst)

5. 
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

6. WHO CLASSIFICATION 2005

1) Ossifying Fibroma (OF)
2) Fibrous Dysplasia
3) Osseous Dysplasia
a. Periapical Osseous Dysplasia
b. Focal Osseous Dysplasia
c. Florid Osseous Dysplasia
d. Familial Gigantiform Cementoma
4)Central Giant Cell Granuloma
5)Cherubism
6)Aneurismal Bone Cyst
7)Solitary Bone Cyst

7. 
EVERSOLE 2008 CLASSIFICATION
1.Bone dysplasias
a. Fibrous dysplasia
i. Monostotic
ii. Polyostotic
iii. Polyostotic with endocrinopathy (McCune-Albright)
iv. Osteofibrous dysplasia
b. Osteitis deformans
c. Pagetoid heritable bone dysplasias of childhood
d. Segmental odontomaxillary dysplasia
2. Cemento-osseous dysplasias
a. Focal cemento-osseous dysplasia
b. Florid cemento-osseous dysplasia
3.Inflammatory/reactive processes
a. Focal sclerosing osteomyelitis
b. Diffuse sclerosing osteomyelitis
c. Proliferative periostitis
4. Metabolic Disease: hyperparathyroidism

8. 
5. Neoplastic lesions (Ossifying fibromas)
a. Ossifying fibroma
b. Hyperparathyroidism jaw lesion syndrome
c. Juvenile ossifying fibroma
i. Trabecular type
ii. Psammomatoid type
d. Gigantiform cementomas

9. TERMINOLO
GY

• Skeletal developmental anomaly of the bone - defect in osteoblastic
differentiation and maturation
• 2.5% of all bony tumours & 7% of all non-malignant tumours of bone
• Von recklinghausen 1891- osteitis fibrosa cystica.
• Fibrous dysplasia of bone.
Lichentenstein
1938

10. DEFINITION
 A benign lesion, presumably developmental in nature, characterized by
the presence of fibrous connective tissue with a characteristic whorled
pattern and containing trabeculae of immature non lamellar bone.
Waldron 1985.
 Reeds definition : fibrous dysplasia is an arrest of bone maturation in
woven bone with ossification resulting from metaplasia of a non specific
fibro-osseous type.
FIBROUS DYSPLASIA


11. Idiopathic
Non hereditary
Caused by mutation in GNAS1 gene
ETIOLOGY & PATHOGENESIS


12. MOLECULAR PATHOGENESIS

Mutated GNAS
Encodes G-protein
Activation
c-AMP
Elevtion of c-AMP & stimulation of endocrine receptors
Activation
C-fos (proto-oncogene)

13. JAFFE-LICHENSTEINSYNDROME
MCCUNE ALBRIGHT
SYNDROME

14. 
Mutated osteoblasts
Over expression IL-6
Stimulate osteoclastic activity
Bone Lesion Expansion

15. Monostotic form

Polyostotic form
Craniofacial form
CLINICAL FEATURES
THREE DISEASE PATTERNS ARE
RECOGNIZED

16. 
70%-80% of fibrous dysplasia.
Occurs in rib, femur , tibia, craniofacial
bones and humerus
Pain or pathologic fracture
Bone deformity less severe
Painless swelling of the jaw
Swelling involves labial or buccal plate
Protuberance - inferior border of mandible
MONOSTOTIC FORM

17. 
MANDIBULAR
LESIONS- TRULY
MONOSTOTIC.
clinical term ‘leontiasis ossea’ –FD of maxilla or
facial bones & give the patient a leonine
appearance

18. 
20%-30% of fibrous dysplasia.
Sites: Femur, tibia, pelvis, ribs, skull and facial
bones, upper extrimites, lumbar spine &
clavicle.
Tends to occur in unilateral distribution.
Involvement asymmetric and generalized on
bilateral lesions.
POLYOSTOTIC FORM

19. 
 Equal in males & females.
 Commonly-3-15yrs
 Polyostotic-asymptomatic before 10 years .
 Monostotic-asymptomatic -20-30 years.

20. 
Pain in
involved limb
Spontaneous
fracture
Structural
integrity
weak
Bowing of
weight
bearing bones
curvature of
femoral neck
Shepherd’s
crook
deformity

21. MALALIGNM
ENT TIPPING
DISPLACEME
NT
Intact over lesion
Maxillary sinus, zygomatic process
Floor of orbit, extend to base of
skull

22. • Polyostotic FD and lesions of skin
or café-au-lait spots

Jaffe Lichenstien
syndrome
• Polyostotic FD, café-au-lait spots
and endocrine disturbances
Mc-Cune
Albright
syndrome
• Fibrous dysplasia & intramuscular
myxoma
Mazabraud
syndrome

23. CAFÉ-AU-LAIT
PIGMENTATION
Fibrous dysplasia Neurofibroma

24. 
CAFE-AU-LAIT
SPOTS
MAZABRAUDS
SYNDROME
MALIGNANCIES
• Increased
melanin in basal
cells of epidermis
• Cutaneous
pigmentation
seen ipsilateral to
side of bone
lesion.
• Fibrous dysplasia
and
intramuscular
myxoma, risk of
sarcomatous
malformation
• Osteosarco Mc-
Cune albright
syndrome
• Chondrosarco
Mc-Cune albright
syndrome
• Fibrosarco Mc-
Cune albright
syndrome
• LiposarcoMc-
Cune albright
syndrome

25. 
In 10-25% of pt. with monostotic form.
In 50% of pt. with polyostotic form.
Also in isolated craniofacial form.
No extracranial lesions present.
Sites: frontal, sphenoid, maxillary, ethmoid
bones.
Extreme prominence of zygomatic process,
facial deformity
Vestibular dysfunction, tinnitus, hearing loss,
Hypertelorism, cranial asymmetry, facial deformity,
visual impairment, exophthalmos, blindness .
CRANIOFACIAL FIBROUS
DYSPLASIA

26. Network of fine bone trabec

ulae
Increased trabeculation – lesion more opaque & mottled
appearance
Cortical bone becomes thinned
Roots of teeth moved out of normal position
RADIOGRAPHIC FEATURES

27. • Radiolucent
image
• Ground glass/
Orange peel
appearance
• Radiopaque
image
Rind
sign

28. 
HISTOLOGIC FEATURES
 Proliferating fibroblasts in a compact stroma of interlacing
collagen fibres.
 Irregular bony trabeculae scattered throughout lesion.
 Chinese character shaped.
 Trabeculae usually coarse woven bone
 Lesions rich in spindle shaped fibroblasts with a swirled
appearance within the marrow space
 Lesional bone fuses directly with normal bone at the periphery

29. 10x
Bony trabeculae
Fibrous
connective tissue
osteocyte
peri-trabecular cleft

30. WOVEN BONE
Lamellar bone
Monotonous pattern-calcification-FD differs from haphazard mixture of woven,
lamellar bone & spheroid particles-ossifying fibroma & cemento-osseous dysplasia.

31. 
FIBROUS
DYSPLASIA
 1st & 2nd decades
 Usually-Maxilla
 Self limited
 One or more bones
 Diffuse opacity
 Vascular matrix
 Woven bone trabeculae
 Chinese letter pattern
 Peri-trabecular clefting is
present
Ossifying fibroma
 3rd & 4th decades
 Usually-mandible
 Continuous
 One bone
 Circumscribed
 Cellular fibrous matrix
 Bony islands & trabeculae
 ”bizarre” character shape
 Absent

32. No significant change in se
rum
calcium/phosphorus
Elevated Alkaline phosphatase
Moderate increase in Basal Metabolic Rate
LAB FINDING

33. DIFFERENTIAL
DIAGNOSIS

Ossifying Fibroma
Periapical Cement-Osseous Dysplasia
Focal Cement-Osseous Dysplasia
Florid Cement-Osseous Dysplasia
Paget’s disease
Osteosarcoma
Hyperparathyroidism
Chronic osteomyelitis

34. Conservative treatment-prevent
deformity.
Management requires a multidisciplinary approach -polyostotic.
Bisphosphonate therapy may help to improve function, decrease pain,
& lower fracture risk -some patients.
Surgery-complete resection, restoring function &improving facial
aesthetics
TREATMENT

35.  0.4% - 4%
 Osteosarcoma
 Fibrosarcoma
 Chondrosarcoma
 28% - seen in radiated-Radiotherapy contraindicated ….
MALIGNANT
TRANSFORMATION


36. Prognosis-good
Although-bad outcomes-more frequently among young
patients or with polyostotic forms.
PROGNOSIS


37. CONCLUSION

•  FIBROUS DYSPLASIA IS A LESION OF BONE COMMONLY
AFFECTING THE YOUNGER
• AGE GROUP. IT SHOWS SIMILARITIES WITH
OTHER FIBRO OSSEOUS LESIONS CLINICALLY,
RADIOLOGICAL & HISTOPATHOLOGICALLY.
•  HENCE THROUGH KNOWLEDGE ABOUT THESE LESIONS IS
NECESSARY FOR PROPER DIAGNOSIS & TREATMENT PLAN.

38. 
 Shafer’s textbook of oral pathology 7th Edition
 Burkitt’s oral medicine,11th edition.
 Neville, Damm, Allen, Bouquot. Oral & maxillofacial
pathology ,1st south asia edition
 Lucas Pathology Of Tumours Of The Oral Tissues
 Fibrous Dysplasia. Pathophysiology, Evaluation, and
Treatment www.jbjs.org
REFERENCES

39. 
polyostotic Fibrous Dysplasia-
clinical dentistry jul-sep-2010/vol
 Craniofacial
contemporary
1/issue 3.
 Fibrous Dysplasia In the Maxillomandibular region–
Journal of IMAB - Annual Proceeding (Scientific
Papers) vol. 16, book 4, 2010
 Regezi- Textbook of oral pathology- 5th edition.

40. THANKYOU