FIBRO-OSSEOUS LESIONS

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
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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.

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CLASSIFICATION
FOL of medullary bone origin

FOL of PDL origin

FD Fibroma Fibro osteoma CF Cherubism OF Juvenile OF COF Giant cell tumor ABC Jaw lesions in hyperparathyroidism Pagets disease
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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.
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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
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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
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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
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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.

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Polyostotic FD

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

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

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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.
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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

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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.

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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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