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FIBRO-OSSEOUS LESIONS

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Title: FIBRO-OSSEOUS LESIONS


1
FIBRO-OSSEOUS LESIONS
dr shabeel pn
2
INTRODUCTION
  • The term FOL is a generic designation of a
    group of bone disorders characterized by the
    replacement of bone by a benign connective tissue
    matrix that displays varying degrees of
    mineralization in the form of woven bone or
    cementum.
  • Group include
  • developmental lesions
  • reactive / dysplastic lesions
  • neoplastic lesions

3
Importance of Specific Diagnosis
  • The histopathology of all FOL is identical,
    although they range widely in clinical behavior.
  • More specific diagnosis is important because the
    treatment of these pathoses varies from none to
    surgical recontouring to complete removal.

4
CLASSIFICATION
  • FOL of medullary bone origin
  • FD
  • Fibro osteoma
  • Cherubism
  • Juvenile OF
  • Giant cell tumor
  • ABC
  • Jaw lesions in hyperparathyroidism
  • Pagets disease
  • FOL of PDL origin
  • Fibroma
  • CF
  • OF
  • COF

5
WHO classification of Odontogenic tumors (2nd ed,
1992)
  • (a) Fibrous dysplasia
  • (b) Cemento ossifying fibroma
  • - Spectrum of COF CF-COF-OF
  • - Juvenile Ossifying Fibroma
  • WHO type
  • Psammous type
  • (c) Cemento-osseous dysplasia
  • -PCOD
  • -Focal COD
  • -Florid COD
  • -Familial gigantiform cementoma.

6
Modified WHO classification(Speight and Carlos)
  • Fibrous dysplasia
  • Monostotic
  • Polyostotic
  • Craniofacial

Osseous dysplasias PCOD Focal COD Florid
COD Familial gigantiform cementoma
Ossifying fibromas Conventional ossifying
fibroma Juvenile trabecular (WHO type)
OF Juvenile psammomatoid OF
7
FIBROUS DYSPLASIA
  • Developmental or hamartomatous condition
  • Unknown etiology
  • Characterized by proliferation of cellular
    fibrous connective tissue mixed with bony
    trabeculae
  • Sporadic condition, resulting from post zygotic
    mutation in GNAS 1 gene
  • Clinical severity depends on the point of time
    during embryonic, fetal or post natal life at
    which mutation of GNAS 1 occurs

8
Clinical Features
  • Monostotic fibrous dysplasia
  • Limited to single bone
  • 80 85 of all cases
  • Jaws among most common sites
  • Diagnosed during second decade
  • No sex predilection
  • Painless swelling most common feature.
  • Slow growth, become static with skeletal growth
    completion

9
  • Craniofacial fibrous dysplasia
  • Peculiar form affecting skull bones
  • Not restricted to single bone, but confined to
    single anatomic site.
  • Primarily affect maxillae, but may cross sutures
    into sphenoid, zygoma, frontonasal bones and base
    of skull.

10
  • Polyostotic FD
  • Involvement of two or more bones other than
    craniofacial bones
  • Number of bones a few to 75 of skeleton
  • With café au lait (coffee with milk)
    pigmentation, Jaffe Lichtenstein syndrome
  • With café au lait pigmentations and multiple
    endocrinopathies sexual precocity, pituitary
    adenoma or hyperthyroidism, McCune Albright
    syndrome

11
  • May present with facial asymmetry
  • Clinical features usually dominated by symptoms
    related to long bone lesions Pathologic
    fractures
  • Length discrepancy due to involvement of upper
    portion of femur (hockey stick deformity)
  • Café au lait pigmentation generally unilateral
    tan macules on the trunk and thighs.
  • - May be congenital
  • - Oral cavity can be involved
  • - Margin typically irregular (Coastline of Maine)

12
Radiographic Features
  • Site Most often involves maxilla
  • Posterior aspect. Unilateral
  • Periphery ill defined. Gradual blending
  • Internal structure.
  • Variation pronounced is maxilla
  • More uniform in mandible

13
  • Radiolucent
  • Mixed radiolucent-radiopaque
  • Heterogenous pattern
  • Orange peel pathognomonic
  • Ground glass
  • Radiopaque cottonwool or diffuse

14
  • Effect on surrounding structures
  • Thinning of cortex
  • Displacement of antral walls
  • Loss of lamina dura
  • Displacement of teeth
  • Interference with normal eruption
  • Inferior alveolar canal displaced superiorly /
    inferiorly
  • Superior displacement unique to FD.

15
CT
  • To define extent of involvement of cranial base.
  • 34 513 HU
  • Heterogeneous pattern of CT densities associated
    with scattered or confluent islands of bone
    formation

16
MRI
  • Intermediate signal on T1 weighted and proton
    weighted images
  • Heterogenous hypointense signal of T2 weighted
    scan
  • Moderate to significant contrast enhancement
    after i.v. Gd contrast infusion.

17
Management and Prognosis
  • Small lesions can be resected entirely
  • Most lesion stabilize with skeletal maturation
  • Surgical recontouring after skeletal maturation
  • Osteosarcoma-especially in those who received
    radiation.
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