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FIBRO-OSSEOUS LESIONS. 2Dr.Haris PS/OMR INTRODUCTION The term FOL is a generic designation of a group of bone disorders characterized by the replacement.

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Presentation on theme: "FIBRO-OSSEOUS LESIONS. 2Dr.Haris PS/OMR INTRODUCTION The term FOL is a generic designation of a group of bone disorders characterized by the replacement."— Presentation transcript:

1 FIBRO-OSSEOUS LESIONS

2 2Dr.Haris PS/OMR 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. 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 3Dr.Haris PS/OMR 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 4Dr.Haris PS/OMR CLASSIFICATION FOL of medullary bone origin FD FD Fibro osteoma Fibro osteoma Cherubism Cherubism Juvenile OF Juvenile OF Giant cell tumor Giant cell tumor ABC ABC Jaw lesions in hyperparathyroidism Jaw lesions in hyperparathyroidism Pagets disease Pagets disease FOL of PDL origin Fibroma Fibroma CF CF OF OF COF COF

5 5Dr.Haris PS/OMR WHO classification of Odontogenic tumors (2 nd 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 6Dr.Haris PS/OMR Modified WHO classification (Speight and Carlos) Fibrous dysplasia MonostoticPolyostoticCraniofacial Osseous dysplasias PCOD Focal COD Florid COD Familial gigantiform cementoma Ossifying fibromas Conventional ossifying fibroma Juvenile trabecular (WHO type) OF Juvenile psammomatoid OF

7 7Dr.Haris PS/OMR FIBROUS DYSPLASIA Developmental or hamartomatous condition Developmental or hamartomatous condition Unknown etiology Unknown etiology Characterized by proliferation of cellular fibrous connective tissue mixed with bony trabeculae Characterized by proliferation of cellular fibrous connective tissue mixed with bony trabeculae Sporadic condition, resulting from post zygotic mutation in GNAS– 1 gene 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 Clinical severity depends on the point of time during embryonic, fetal or post natal life at which mutation of GNAS – 1 occurs

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

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

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

11 11Dr.Haris PS/OMR May present with facial asymmetry May present with facial asymmetry Clinical features usually dominated by symptoms related to long bone lesions – Pathologic fractures 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) 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. 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 12Dr.Haris PS/OMR Radiographic Features Site – Most often involves maxilla Site – Most often involves maxilla Posterior aspect. Unilateral Posterior aspect. Unilateral Periphery ill defined. Gradual blending Periphery ill defined. Gradual blending Internal structure. Internal structure. Variation pronounced is maxilla More uniform in mandible

13 13Dr.Haris PS/OMR Radiolucent Radiolucent Mixed radiolucent-radiopaque Mixed radiolucent-radiopaque Heterogenous pattern Orange peel – pathognomonic Ground glass Radiopaque – cottonwool or diffuse Radiopaque – cottonwool or diffuse

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

15 15Dr.Haris PS/OMR CT To define extent of involvement of cranial base. To define extent of involvement of cranial base. 34 – 513 HU 34 – 513 HU Heterogeneous pattern of CT densities associated with scattered or confluent islands of bone formation Heterogeneous pattern of CT densities associated with scattered or confluent islands of bone formation

16 16Dr.Haris PS/OMR 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 17Dr.Haris PS/OMR Management and Prognosis Small lesions can be resected entirely Small lesions can be resected entirely Most lesion stabilize with skeletal maturation Most lesion stabilize with skeletal maturation Surgical recontouring after skeletal maturation Surgical recontouring after skeletal maturation Osteosarcoma-especially in those who received radiation. Osteosarcoma-especially in those who received radiation.


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