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• 1. In The Name Of GOD

2. Bone pathology 3. ( ) ( ) 4. opalescent( . ) 5. Deformity Bone fractures Wormian bones in skull Opalescent teeth Shell teeth Class III maloclusion Clinical and radiographic features: 6. Type I 10% (10% ) 30 Type II 90% 4 7. Type III Type IV 50% 8. OSTEOPETROSIS 9. . . 10. Symptoms Fractures Cranial nerve palsies Hepatosplenomegaly Anemia Frequent infections Blindness Deafness Enlarged spleen Bleeding Headaches 11. Malignant osteopetrosis Normocytic anemia &hepatosplenomegaly Granulocytopenia Facial deformity Cranial nerve compression Delayed eruption of tooth Pathologic fracture osteomyelitis Benign osteopetrosis 40% asymptomatic Pathologic fracture Osteomylitis Cranial nerve compression infantile adult 12. Pathology: . Marked thickening of bone cortices and trabeculae with marked narrowing of marrow spaces . Narrowing of neural foramina with compression of exiting nerves 13. Defect in CBFA1 gene Absent of clavicles Craniofacial malformasion Wormian bone of skull Cleft palate Failure of eruption of permanent teeth Supernumerary teeth Mandibular prognathism Lack of secondary cementum 14. PAGET'S DISEASE OF BONE (OSTEITIS DEFORMANS) . ( ). 15-40 . 15. 40 . ( ) . 16. . 20% 1/3 (. ) alkaline phosphatase 113% 17. . (circumscripta) . black beard lincoln . 18. .1 .2 19. reversal line)) . 20. . 40 . . . . 21. 60 30. 70 . Nonaggressive lesion: . Aggressive lesion: Central Giant cell Granuloma 22. . 20 . . . 23. Giant cell 24. 4 2-5 . CGCG. Cherubism 25. cufflike ( .) 26. - . 27. 1020 . . () 28. 29. Aneurysmal bone cyst Rare in jaws Jaw lesions are usually in the mandibular ramus and angle Affect patients usually between 10 and 20 years Are of unknown etiology Form very expansile soap-bubble radiolucencies which may be mistaken clinically and radiografically for ameloblastoma Histologically consist of a mass of blood-filled spaces with scattered giant cells (blood soaked spongy ) Are treated by curettage but sometimes recure 30. : . . 31. Fibrous dysplasia: . GNASI. . . . 32. 80% craniofacial f.d 33. ( 75% ) . 34. ground-glass( . ground-glass ) 35. : . 36. . . Mazabraud . 37. . PDL . 38. 36 . . 39. cementoma (10-141) 3050 20 . . PDL. 40. simple bone cyst 41. : - . 42. 34 Juvenile o.f 15 43. : . 44. Fibrous dysplasia < Ossifying fibroma < 45. Benign, second decade; males (3:1) Painful (high prostaglandin levels in lesion) Central nidus of vascular spaces surrounded by interlacing reactive trabecular bone. Therapy - Resection of nidus - bone graft. 46. Central hemorrhagic nidus surrounded by dense rim of sclerotic bone 47. Histopathologic feature Nidus contains interlacing network of osteoid and bony trabeculae with variable amount of mineralization, lying in vascular fibrous tissue 48. Clinically similar to osteoid osteoma (large) Common location -- vertebral column Histology similar but rare nidus Can be locally aggressive Therapy - curettage/resection with bone graft 49. 80% 30 24 cm 2cm 50. : reversal . 51. Osteomas are benign tumors composed of mature compact or cancellous bone Osteomas are essentially restricted to the craniofacial skeleton The common palatal and mandibular tori and buccal exostoses periosteal osteoma, or endosteal osteoma detected in young adults and are generally asymptomatic, solitary lesions 52. Histopathologic Features: Compact osteomas are composed of normal- appearing dense bone showing minimal marrow tissue Cancellous osteomas are composed of trabeculae of bone and fibrofatty marrow. Osteoblastic activity may be fairly prominent 53. 54. 55. ollier: :Maffucci low grade . 56. ( ) 75% 2/3 . 57. ( ) . (1-2-3- ) :2 (10-15) 50 1015 (33) 58. 33 1015 . . 59. sunbrust or sunray 25% . 60. . 61. resorption of the roots of teeth involved by the tumor(spiking" resorption) 62. Histopathologic Features direct production of osteoid by malignant mesenchymal Cells Osteoblastic Chondroblastic Fibroblastic Chondroblastic osteosarcomas constitute a substantial proportion of all osteosarcomas of the jaws 63. . . 64. ( ) . 65. . . 66. . . . . . 67. CHONDROSARCOMA Chondrosarcoma is a malignant tumor characterized by the formation of cartilage, but not bone, by the tumor Cells Clinical and Radiographic Findings: chondrosarcoma is primarily a neoplasm of adulthood(6-7decay) Chondrosarcomas arise most frequently in the maxilla A painless mass or swelling is the most common presenting Sign Radiographically, the tumor usually shows features suggestive of a malignancy, consisting of a radiolucent process with poorly defined borders Root resorption or symmetric widening of the periodontal ligament space of the teeth involved by the tumor also may be noted 68. . . . . 69. Histopathologic Features Chondrosarcomas are composed of cartilage showing varying degrees of maturation and cellularity Typical lacunar formation within the chondroid matrix is visible, although this feature may be scarce in poorly differentiated tumors The tumor often shows a lobular growth pattern, with tumor lobules separated by thin fibrous connective tissue septa 70. . . . 71. 1: . . 2: . 3: . 72. Ewing sarcoma : T(11;22) ESR 73. : 75% CD99-MIC2 : : 74. 14 1000 .0/2% 7000 1-2-3- de nevo 75. 2/3 1/2 1/3 . : 76. (Numb chin syndrom) mixed. poorly. 77. THANKS FOR YOUR ATTENTION