Fibrousdysplasia

FIBROUS DYSPLASIA

1. FIBROUS DYSPLASIA PRESENTED BY Dr. D.Venkatesh Kumar 2 nd yr PG

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

3. INTRODUCTION

4. WHO CLASSIFICATION 1992 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 Ossifying Fibroma (OF) Fibrous Dysplasia 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. Terminology 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. ETIOLOGY & PATHOGENESIS

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

13. JAFFE-LICHENSTEIN SYNDROME MCCUNE ALBRIGHT SYNDROME

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

15. CLINICAL FEATURES Three disease patterns are recognized

16. MONOSTOTIC FORM

17. Mandibular lesions- truly monostotic . clinical term ‘leontiasis ossea ’ –FD of maxilla or facial bones & give the patient a leonine appearance

18. POLYOSTOTIC FORM

19. E qual in males & females. Commonly-3-15yrs Polyostotic -asymptomatic before 10 years . Monostotic -asymptomatic -20-30 years.

20. Café-au- lait pigmentation Fibrous dysplasia Neurofibroma

21. Craniofacial Fibrous Dysplasia

22. RADIOGRAPHIC FEATURES

23. Radiopaque image Rind sign

24. 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

25. 10x Bony trabeculae Fibrous connective tissue osteocyte peri - trabecular cleft

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

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

28. LAB FINDING

29. Differential Diagnosis Chronic osteomyelitis

30. TREATMENT

31. 0.4% - 4% Osteosarcoma Fibrosarcoma Chondrosarcoma 28% - seen in radiated- Radiotherapy contraindicated …. Malignant transformation

32. PROGNOSIS

33. 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.

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

35. Craniofacial polyostotic Fibrous Dysplasia-contemporary clinical dentistry jul-sep-2010/ vol 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- 5 th edition.

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