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Bone pathology

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Bone Pathology

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Bone pathology

  1. 1. Bone Pathology Dr. Arsalan Malik Assistant Professor (Oral Pathology) ِ‫ح‬َّ‫الر‬ ِ‫من‬ْ‫ح‬َّ‫الر‬ ِ‫هللا‬ ِ‫م‬ْ‫س‬ِ‫ب‬ِ‫يم‬ 1
  2. 2. Osteogenesis Imperfecta  It is a heterogeneous group of heritable diseases characterized by impairment of collagen maturation.  Collagen forms a major portion of bone, dentine, sclerae, ligaments and skin  Osteogenesis imperfecta imparts problems in every structure made by collagen  Abnormal collagen maturation results in thin cortex, fine trabeculation and diffuse osteoporosis  upon fracture, healing will occur but may be associated with excessive callus formation 2
  3. 3. Clinical & Radiographic Features  Blue sclerae, altered teeth, hypoacusis, long bone, spine deformities and joint hyperextensibility  Radiographs show deformation of the long bones, multiple fractures and wormian bones in the skull. 3
  4. 4. Clinical & Radiographic Features4
  5. 5. Oral Manifestations Both dentitions are involved showing blue translucence Premature pulpal obliteration Class III malformations Multifocal radiolucencies Mixed radiolucencies and radio-opacities 5
  6. 6. Oral Manifestations6
  7. 7. Types of Osteogenisis Imperfecta  Type I  Type II  Type III  Type IV 7
  8. 8. Type I Osteogenisis Imperfecta  Most common and mildest form  Mild to moderate bone fragility  Most fractures occur during preschool time  Hearing loss develops before 30  Sclerae is blue and help in diagnosis  Hypermobile joints and easy bruising 8
  9. 9. Type II Osteogenesis Imperfecta Most severe form Exhibits extreme bone fragility and frequent fracture  many patients are still born and 90 % die before 4 week of age Opalescent teeth may be present 9
  10. 10. Type III Osteogenisis Imperfecta  Moderately severe to sever bone fragility  Sclerae is pale yellow or blue and fades as the child grows  Hearing loss and ligament hyperextensibility present  Many have normal teeth and some have opalescent teeth 10
  11. 11. Type IV Osteogenisis Imperfecta  Mild to moderately severe bone fragility  Sclera may be blue but fades with age  In 50 % of patients fractures are present at birth  Many have normal teeth and some have opalescent teeth  Frequency of fracture decreases with age 11
  12. 12. Histopathological Features  Bone architecture remains immature throughout life  Failure of woven bone to become transferred to lamellar bone  Teeth show abnormalities of dentine similar to dentinogenisis imperfecta  Teeth appear opalescent 12
  13. 13. Treatment  There is no cure for this, symptomatic treatment is the only choice  Management of fractures is a major problem  Mainstay of treatment is physiotherapy, rehabilitation and orthopaedic surgery  Medical treatment with Bisphosphonates provides relief from pain but its long term use is not recommended  In patients with severe malocclusion, orthognathic surgery is recommended 13
  14. 14. Osteopetrosis (Marble Bone Disease)  It is a group of hereditary skeletal disorders characterized by marked increase in bone density resulting from a defect in remodelling caused by failure of normal osteoclast formation.  Number of osteoclasts is normal but function is defective  Continuous bone formation and defective resorption results in thickening of bone. 14
  15. 15. Clinical & Radiographic Features Infantile Osteopetrosis  Patients having disease at birth or early infancy usually have severe disease called malignant osteopetrosis  Facial deformity, broad face, hypertelorism, snub nose, frontal bossing.  Narrowing of arches due to delayed eruption is common  In dental radiographs roots of teeth are often invisible due to increased density of bone 15
  16. 16. Clinical & Radiographic Features Adult Osteopetrosis  It is discovered later in life and exhibits less severe manifestations  Inherited as autosomal dominant trait and termed benign osteopetrosis  Approx 40 % of patients are symptom free and long bones are not effected.  Often It is diagnosed by increased bone opacity in dental radiographs  Frequent fractures and cranial nerve compression are common 16
  17. 17. Clinical & Radiographic Features 17
  18. 18. Histopathological Features  Tortuous lamellar trabeculae replacing the cancellous bone  Globular amorphous bone deposition in the marrow space  Osteophytic bone formation 18
  19. 19. Treatment & Prognosis  Adult Osteopetrosis is mild in severity and is associated with long term survival as compared to infantile type where patients die during 1st decade of life.  Bone marrow transplant is the only hope for treatment  Supportive measures include transfusion, antibiotics, drainage and surgical debridement 19
  20. 20. Cleidocranial Dysplasia  It is best known for its dental & clevicular abnormalities  It is caused by the mutation in gene that is responsible for osteoblastic differentiation and bone formation 20
  21. 21. Clinical & Radiographic Features  Mainly the clevicles and skull are effected however other bones may be involves. Clevicles show varying degree of hypoplasia and malformation  In 1 % of patients, clevicles are absent  Patient’s appearance is diagnostic (Large head and frontal bossing)  skull radiographs show delayed suture closure or wide open throughout life 21
  22. 22. Clinical & Radiographic Features22
  23. 23. Histopathological Features  Insufficient bone resorption is the reason for impaired tooth eruption  Some theories suggested impaired cementum formation as a main factor but it is not proved 23
  24. 24. Clinical & Radiographic Features Patients often have a narrow high arched palate and increased chances of cleft palate Abnormal spacing in the lower incisors and delayed eruption of teeth Supernumerary teeth are present increased density of mandible and narrow maxillary arch 24
  25. 25. Clinical & Radiographic Features25
  26. 26. Treatment & Prognosis  No specific treatment exists for skull, clevicles and bone abnormalities  Regarding dental problems, full mouth extraction with denture construction, auto-transplantation of impacted teeth with prosthetic restoration  removal of the primary and supernumerary teeth followed by exposure of permanent teeth 26
  27. 27. Focal Osteoporotic Marrow Defect  An area of hematopoitic marrow that is sufficient in size to produce an area of radiolucency which is confused with an intraosseous neoplasm.  It does not represent a pathological process  Reasons may be  Aberrant bone regeneration after tooth extraction  Persistence of fetal marrow  Marrow hyperplasia in response to increased demand for erythrocytes 27
  28. 28. Clinical & Radiographic Features  Asymptomatic and purely an incidental finding  It appears a radiolucent area varying in size  Defect have ill defined borders and fine central trabeculae  No expansion of jaw is observed 28
  29. 29. Histopathological Features  The defects contain cellular hematopoitic or fatty marrow  Lymphoid aggregates may be present  No abnormal osteoblastic / osteoclastic activity 29
  30. 30. Treatment & Prognosis  Incisional biopsy often is necessary to establish diagnosis  No treatment is necessary as it is not a pathological condition 30
  31. 31. Paget’s Disease of Bone  It is characterized by abnormal and anarchic resorption & deposition of bone resulting in weakening and distortion of affected bones  Inflammatory, genetic and environmental factors may play a role in this disease 31
  32. 32. Clinical Features  Men > Women  Whites > Blacks  Older > younger  Asymptomatic disease is usually diagnosed in radiographs taken for other reasons or serum alkaline phosphatase levels.  Its frequency increases with age 32
  33. 33. Clinical Features  Disease may be monostotic (limited to one bone) or polyostotic (multiple bones involved)  Bone pain is a frequent complaint  Pegetic bone often forms near joints and promotes osteoarthritic changes with associated joint pain and limited mobility 33
  34. 34. Clinical Features  Jaw involvement is present in almost 17 % of cases  Maxillary involvement results in mid face enlargement  Nasal obliteration, enlarged turbinates, obliterated sinuses and deviated septum  Alveolar ridges become enlarged and create problems for denture wearers 34
  35. 35. Radiographic Features  Initial stages show decreased radiodensity of bone and alteration of the trabecular pattern.  During bone formation patchy areas of sclerotic bones formed giving a cotton wool appearance in radiographs  Teeth often exhibit hypercementosis 35
  36. 36. Histopathological Features  Apparent uncontrolled alternating resorbtion and deposition of bone.  In active resorptive stage numerous osteoclasts surround the trabeculae  ‘’Reversal lines’’ mark the junction between alternating resorptive and formative phase  A highly vascular fibrous connective tissue replaces the bone 36
  37. 37. Diagnosis  Patients show raised levels of serum alkaline phosphatase but normal levels of calcium and phosphate  Serum alkaline phosphatase is considered the most specific marker of bone formation  Urinary hydroxyproline levels often are markedly elevated  Lab parameters and clinical examination is sufficient for diagnosis 37
  38. 38. Treatment & Prognosis  Asymptomatic patients usually do not need treatment  In symptomatic cases, bone pain is controlled by acetaminophen or NSAIDs  Neurological complications are due to pressure of bony structures on cranial nerves. Use of Parathormone (PTH) antagonists can reduce bone turnover and improve the biochemical parameters.  Dental patients may require new dentures due to increased size of alveolar ridges. 38
  39. 39. Central Giant Cell Granuloma  It is considered to be a non neoplastic lesion  60 % cases arise before 30 years  Females > males  70 % cases arise in mandible  Anterior portion > posterior 39
  40. 40. Clinical Features  Some cases are asymptotic and diagnosed in routine radiographs  Symptomatic cases represent pain, paraesthesia or perforation of cortical plate resulting in ulceration of overlying mucosa  Nonaggressive lesion  Exhibit few or no symptoms, slow growth, no cortical perforation / root resorption  Aggressive lesions  Pain. Rapid growth, cortical perforation, tendency to recur 40
  41. 41. Radiographic Features  Radiolucent defects which may be unilocular / multilocular  Range from 5 mm to 10 cm in diameter  D/D of unilocular lesions  Perioapical granuloma  Periapical cyst  D/D of multilocular lesions  ameloblastoma 41
  42. 42. Histopathological Features  Multinucleated giant cells in back ground of ovoid mesenchymal cells & round monocyte-macrophages  Stroma is loosely arranged and edematous  Areas of erythrocyte extravasation and hemosiderin deposition are prominent  Foci of osteoid and newly formed bone are occasionally present in lesion 42
  43. 43. Histopathological Features43
  44. 44. Treatment & Prognosis  Usually treated by thorough curettage  Aggressive lesion show more tendency to recur  They require radical surgery for cure  Three alternatives to surgery are  Corticosteroids  Calcitonin  Interferon alfa-2a 44
  45. 45. Cherubism  Rare developmental disorder generally inherited as autosomal dominant trait  Males > females 45
  46. 46. Cherubism  It occurs due to spontaneous mutation in gene that codes for protein responsible for activity of osteoblasts & osteoclasts during bone formation  The term Cherubism is used because of pulmp-cheeked little angles depicted in some paintings 46
  47. 47. Clinical Features  Usually occur between 2-5 years of age  Clinical alterations progress till puberty then stabilize and slowly regress  Bilateral involvement of the posterior mandible that gives chubby cheeks  ‘’Eye upturned to heaven’’ appearance due to wide rim of sclera noted below the iris  Marked cervical lymphadenopathy 47
  48. 48. Clinical Features  Mandibular lesions typically appear as painless, bilateral expansion of the posterior mandible  Bilateral expansion is symmetrical  Maxillary tuberosity area may be involved  Widening and distortion of alveolar arches  Failure of eruption, impair mastication, speech difficulties, loss of normal vision 48
  49. 49. Radiographic Features  Multilocular, expansile radiolucencies  Appearance is virtually diagnostic because of bilateral location  It typically involves the jaws but ribs & humerus may be involved 49
  50. 50. Histopathological Features  Lesional tissue consist of vascular fibrous tissue containing variable number of multinucleated giant cells  Giant cells are small and arranged focally  Foci of extravasated blood is common  Stroma tends to be loosely arranged  In older resolving lesions, tissues become more fibrous, the number of giant cells decreases and new bone formation is seen. 50
  51. 51. Treatment  In many cases lesion is self limiting after puberty  In some patients the recovery is very slow and in some the deformity may persist  In some cases surgical curettage may result in early recovery and in some cases it worsens the situation  Radiation therapy is contraindicated because of risk of development of postirradiation stroma 51
  52. 52. Fibro-Osseous Lesions of the Jaws  It is a diverse group of processes that are characterized by replacement of normal bone by fibrous tissues containing a newly formed mineralized product.  Fibrous Dysplasia  Cemento-Osseous Dysplasia  Focal Cemento-Osseous Dysplasia  Periapical Cemento-Osseous Dysplasia  Florid Cemento-Osseous Dysplasia  Ossifying Fibroma 52
  53. 53. Fibrous Dysplasia  A developmental tumour like condition characterized by replacement of normal bone by an excessive proliferation of cellular fibrous connective tissue intermixed with irregular bony trabeculae  It may involve one bone, multiple bones with or without cutaneous & endocrine manifestations  Degree of severity depends upon time of mutation 53
  54. 54. Clinical Features Monostotic Fibrous Dysplasia  80 – 85 % of cases  Limited to single bone  Male = females  Jaws are most commonly effected  Maxilla > mandible  Painless swelling of jaws 54
  55. 55. Radiographic Features  Ground glass appearance due to poorly calcified bony trabeculae arranged in a disorganized pattern  In mandible, superior displacement of inferior alveolar canal is observed  In maxilla, sinus floor shifts superiorly 55
  56. 56. Clinical Features Polyostotic Fibrous Dysplasia  Relatively uncommon  Combined with café au lait pigmentation is termed as jaffee-Lichtenstein syndrome  Combined with café au lait pigmentation and endocrinopathies is termed McCune- Albright syndrome 56
  57. 57. Clinical Features Polyostotic Fibrous Dysplasia  Pathological fractures are common  Pain and leg length discrepancy is common  In Mc-Cune-Albright Syndrome, sexual disturbances are observed in females as endocrine manifestations 57
  58. 58. Histopathological Features Irregularly shaped trabeculae of immature bone in a cellular, loosely arranged fibrous stroma Bony trabeculae are not connected to each other and assume a Chinese Script writing appearance Lesional bone directly fuse to normal bone without any capsule Jaw and skull lesions undergoes maturation with time 58
  59. 59. Treatment  Smaller lesions are easily resected without difficulty  Larger lesions are difficult to resect completely  Some lesion tend to regress with time but cosmetic problems are addressed through surgery 59
  60. 60. Cemento-Osseous Dysplasia  Occurs in tooth bearing areas of the jaws and most common fibro osseous lesion in clinical practice  Some believe it arises from periodontal ligament and some believe it arises from defect in extra-ligamentry bone remodelling.  Three types are observed  Focal  Periapical  Folrid 60
  61. 61. Focal Cemento-Osseous Dysplasia  Single site of involvement  3rd – 6th decade  90 % in females  Asymptomatic , smaller than 1.5 cm in diameter  Occur in posterior mandible  Lesion varies from completely radiolucent to dense radio-opaque 61
  62. 62. Periapical Cemento-Osseous Dysplasia  Periapical region of anterior mandible  Female > Males  3rd – 5th decade  Asymptomatic radiolucency covering the roots of several teeth  Lesions tend to mature with time  Does not expand the jaws 62
  63. 63. Florid Cemento-Osseous Dysplasia  Multifocal involvement not limited to anterior mandible  Bilateral symmetrical involvement  May remain asymptomatic or manifest as dull pain and bony expansion  Both edentulous and dentulous areas are involved regardless of presence of teeth 63
  64. 64. Florid Cemento-Osseous Dysplasia64
  65. 65. Histopathological Features  All three patterns of cemento osseous dysplasia demonstrate similar histopathological features  Tissues consist of fragments of cellular mesenchymal tissues composed of spindle shaped fibroblasts and collagen fibres  Free haemorrhage is typically noted interspersed throughout lesion  Within this fibrous connective tissue background is a mixture of woven bone, lamellar bone and cementum like particles  As the lesion matures, the amount of mineralized material increases 65
  66. 66. Histopathological Features66
  67. 67. Diagnosis  In most instances, the distinctive clinical & radiographic picture of periapical & florid types allow a strong preseumptive diagnosis without need of biopsy  In case of focal type. Features are less specific and often require surgical intervention  Findings from surgery are helpful in discrimination between focal cement- osseous dysplasia and ossifying fibroma 67
  68. 68. Treatment  They are not neoplastic so they usually do not need treatment  The best management option for asymptomatic patient is regular follow up & maintaining better oral hygiene to avoid periodontal disease.  In symptomatic patients, antibiotics are given but not effective because of dead sclerotic bone.  Saucerization of the dead bone may speed healing. 68
  69. 69. Ossifying Fibroma  It’s a true neoplasm with significant growth potential  Composed of fibrous tissue containing variable mixture of bony trabeculae and cementutum like spherules.  The cementum like material in the lesion is variation of bone. 69
  70. 70. Clinical Features  Majority of cases are present in 3rd & 4th decade  Female predilection  Mandible > maxilla  Most common in peremolar & molar area  Smaller lesions are asymptomatic but large lesions cause painless swelling of involved bone  Pain & paraesthesia are rarely present 70
  71. 71. Radiographic Features  Well defined and unilocular  May appear completely radiolucent because of calcified material produced in tumour  True lesions are completely radio-opaque with thin radiolucent border.  Root resorption or resorption may be seen 71
  72. 72. Radiographic Features72
  73. 73. Histopathological Features  Lesion is well demarcated from surrounding bone  Some are encapsulated others are not  Tumour is usually submitted in one large or few small pieces  It consists of fibrous tissues of varying cellularity and mineralized material hard tissue portion consist of bone or cementum like material  Intra-lesional haemorrhage is unusual 73
  74. 74. Treatment  Circumscribed nature of lesion allows enucleation of tumour with ease  Large tumours may need surgical resection and bone grafting  Chances of recurrence are very rare 74
  75. 75. Osteoma  Benign tumours composed of mature compact or cancellous bone  These are strictly restricted to craniofacial region  Common palatal & mandibular tori are not considered as osteomas 75
  76. 76. Clinical Features  Osteoms of the jaws arise from surface of the bone or they may be present in medullary bone  Asymptomatic and present in younger age  Body of the mandible, condyle are common locations  Condylar osteoms are considered to be true neoplasms  They disturb the occlusion by deviating the jaw to one side  Facial swelling, pain and limited mouth opening are other signs 76
  77. 77. Radiographic features  They appear as circumscribed sclerotic masses  Periosteal osteomas may exhibit uniform sclerotic pattern or sclerotic margins with central trabeculae  Smaller osteomas are difficult to differentiate from foci of sclerotic bone  The true mature osteomas can be confirmed by observing continued growth 77
  78. 78. Radiographic features78
  79. 79. Histopathological 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 79
  80. 80. Treatment  Larger osteomas of the mandibular body causing symptoms or cosmetic deformity are treated by surgical excision  Smaller asymptomatic osteomas present endosteally do not need treatment but should be observed periodically  Condylar osteomas are surgically removed  Osteomas are completely benign and patients do not experience malignant change 80
  81. 81. Gardner Syndrome  A rare disorder that is inherited as an autosomal dominant trait. Approximately 1/3rd cases represent spontaneous mutations  it is considered to be the part of a spectrum of diseases characterized by colorectal polyposis 81
  82. 82. Clinical Features  The associated colonic polyps develop in 2nd decade of life.  They are adenomatous, they transform into adenocarcinomas.  Common skeletal abnormalities include osteomas  Skull, paranasal sinus and mandible are the commonly affected sites  Patients demonstrate 3 – 6 lesions  Osteomas appear as areas of increased radiodensity  Large osteomas will limit mandibular opening 82
  83. 83. Clinical Features83
  84. 84. Clinical Features  Dental abnormalities include multiple osteomas, multiple impacted teeth and supernumerary teeth  Frequency of extra teeth is low as compared to cleidocrania dyaplasia  Patients show epidermoid cysts of skin  Increased prevalence of thyroid carcinomas is also noted 84
  85. 85. Histopathological Features  Osteomas are compact type  Individual lesion cannot be differentiated from solitary osteomas 85
  86. 86. Treatment & Prognosis  The major problem is malignant transformation of colonic polyps in adenocarcinoma  Prophylactic colectomy is recommended  Removal of jaw osteomas & epidermoid cysts are advised for cosmetic reasons 86
  87. 87. Osteoblastoma & Oteoid Osteoma  These both are closely related tumours that represent identical histopathological features but different clinical behaviours  Osteoid osteoma contain a concentration of peripheral nerves not seen in osteoblastoma  It produces prostaglandins that results in pain which is relieved by prostaglandin inhibitors like asprin.  osteomas are smaller than 2 cm & osteoblastoma is bigger than this 87
  88. 88. Clinical Features (Osteoblastoma)  Most commonly affected bone are vertebral column, sacrum, calvarium, long bones & small bones of hand & foot  Occur before 30 years  Female predominance  Posterior region of mandible  2 – 10 cm  Pain, swelling & tenderness may lead to tooth mobility , root resorbtion and tooth displacement 88
  89. 89. Radiographic Features (Osteoblastoma)  Well defined or ill defined radiolucent lesion often having a patchy areas of mineralization.  Most osteoblastomas arise within the medulary bone however it may present as bony projections from the periosteum of bone ( periosteal osteoblastomas) 89
  90. 90. Clinical Features (Osteoid Osteoma)  Occur in femur, tibia & phalanges  Very rare in jaws & pain is nocturnal & relieved by salicylates  Mostly present out of the jaws (extragnathic lesions) 90
  91. 91. Histopathological Features  Mineralized material that demonstrate reversal lines  At the periphery of large masses there are multinucleated osteoclast like cells and numerous osteoblasts  The supporting stoma consist of loose fibrous connective tissues that contain loose vascular channels  Aggressive osteoblastomas are marked by large epitheliod cells with increased mitotic activity. 91
  92. 92. Treatment  Mostly cases of osteoblastoma & osteoid Osteomas are treated by surgical excision or curettage  Prognosis is good and some lesions will regress even after incomplete excision 92
  93. 93. Cementoblastoma  It is an odontogenic neoplasm of ameloblasts  No gender predilection  Mostly involves premolar & molar area of mandible  Signs of aggressive behaviour like bony expansion, cortical erosion tooth displacement and infiltration into pulp chamber may be observed 93
  94. 94. Radiographic Features  Radio-opaque mass fused to one or more roots and is surrounded by a thin radiolucent rim  The outline and roots of the involved tooth is usually obscured as a result of root resorption & fusion of tumour with tooth 94
  95. 95. Histopathological Features  It resemble osteoblastoma with the primary distinguishing feature of fusion with the involved tooth.  Majority of the tumour consist of sheets and thick trabeculae of mineralized material with irregularly placed lacunae & prominent basophilic reversal lines.  Multinucleated giant cells are often present  Periphery of the lesion is composed of non calcified material which appears as radiolucent rim on radiograph. 95
  96. 96. Histopathological Features96
  97. 97. Treatment  Surgical Extraction of the tumour with involved tooth  Surgical excision of the mass with root amputation and endodontic treatment may be considered.  Total removal of mass with tooth is closely related to chances of recurrence. 97
  98. 98. Osteosarcoma  It is a malignancy of mesenchymal cells that have the ability to produce osteoid or immature bone  Majority of osteosarcomas demonstrate intramedullary origin but a small number may be justracortical 98
  99. 99. Clinical Features  It shows a bimodal age distribution 10 – 20 years and after 50 years  Initial peak occurs during the period of greatest bone growth and involves proximal tibial metaphyses  In older age axial skeleton and flat bones are involved.  Peget’s disease & previous irradiation are associated with increased prevalence 99
  100. 100. Clinical Features  Maxilla = Mandible  In mandible most tumours arise in posterior region  Maxillary tumours usually arise in inferior portion as compared to superior portions  Swelling & pain are the most common symptoms.  Loosening of teeth, paresthesia and nasal obstruction can be seen 100
  101. 101. Clinical Features101
  102. 102. Radiographic Features  It varies from dense sclerosis to a mixed sclerotic and radiolucent lesion to an entirely radiolucent process  Peripheral border of the lesion is ill defined and indistinct making it difficult to distinguish the lesion  The classis sunburst appearance caused by osteophytic bone production on the surface of the lesion is noted in about 25 % of jaw osteosarcomas 102
  103. 103. Radiographic Features103

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