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Edit Comment Close Premium member Presentation Transcript DISEASES OF THE BONES : DISEASES OF THE BONES Prepared by: Dr Sundeep S Bhagwath CLASSIFICATION : CLASSIFICATION Slide 3: A. DEVELOPMENTAL DISEASES: - Cheurbism - Osteopetrosis - Osteogenesis imperfecta - Cleiodocrainal dysplasia B. ENDOCRINAL DISEASES: - Hyperparathyroidism C. IDIOPATHIC DISEASES: - Idiopathic osteosclerosis - Massive osteolysis - Langerhan’s cell disease (Histiocytosis-X) - Paget’s disease Slide 4: D. REACTIVE DISEASES: - Giant cell lesion of bone - Aneurysmal bone cyst - Simple / Traumatic bone cyst E. FIBRO-OSSEOUS LESIONS: (i) Non-neoplastic lesions - - Fibrous dysplasia - Cemento-osseous dysplasia (ii) Neoplasms – - Ossifying fibroma Slide 5: F. INFLAMMATORY DISEASES: - (i) Specific: - Tuberculosis - Actinomycosis (ii) Non specific - Osteomyelitis - Dry socket - Periapical cyst / abscess / granuloma - Osteoradionecrosis Slide 6: G. NEOPLASTIC DISEASES: - (i) Benign: - Osteoma - Osteoid osteoma & osteoblastoma - Chondroma - Chondromyxoid fibroma (ii) Malignant: - Osteosarcoma - Ewing’s sarcoma - Chondrosarcoma DEVELOPMENTAL DISORDERS : DEVELOPMENTAL DISORDERS CHERUBISM : CHERUBISM Rare developmental jaw condition. Transmitted as an autosomal dominant trait. Causes characteristic posterior mandibular swelling due to which the child appears as a plump cheeked angels called “Cherub” in Renaissance paintings. Slide 9: CLINICAL FEATURES: - Age incidence: Children, starting from 1 year Sex incidence: Nil Site predilection: Mostly bilateral involvement Mandible affected more commonly than maxilla. In maxilla, tuberosity region is affected frequently. Slide 10: Signs & symptoms: Begins as painless bilateral expansion of affected bone. Extensive maxillary involvement – skin of upper face stretched – sclera exposed – causes “eyes upturned to heaven” appearance. Slide 11: Progressive, extensive bone involvement causes widening and distortion of alveoli. As a result, developing teeth displaced, fail to erupt. Slide 12: RADIOGRAPHIC FEATURES: - Appear as expansile, multilocular radiolucency. Diagnostic appearance due to the bilateral location. Slide 13: HISTOLOGICAL FEATURES: Features similar to giant cell tumors. Composed of fibrovascular CT containing variable number of multinucleated giant cells. Some cases may show perivascular eosinophilic cuffing. OSTEOGENESIS IMPERFECTA : OSTEOGENESIS IMPERFECTA Most common type of developmental, inherited bone disorder, showing both autosomal dominant and recessive pattern. Comprises heterogeneous group of heritable CT disorder in which bone fragility is the primary feature. Slide 15: CLINICAL FEATURES: - Four types present, each having several subtypes. TYPE I Osteogenesis imperfecta: Commonest type – autosomal dominant. Mild to moderate bone fragility – onset is highly variable – may be present at birth also. Hearing loss develops before 30 years. Some patients may show dentinogenesis imperfecta Slide 16: Deformity of long bones Dentinogenesis imperfecta Slide 17: TYPE II Osteogenesis imperfecta: Extreme bone fragility with frequent fractures. Many patients stillborn – 90% die before 4 weeks of age. Blue sclera present. Dentinogenesis imperfecta present. Slide 18: TYPE III Osteogenesis imperfecta: Moderate to severe bone fragility. Blue sclera present in infants but fades by adulthood. Mortality rate higher in older children. Death from cardiopulmonary complications caused by kyphoscoliosis (backward & lateral curvature of spine). Slide 20: TYPE IV Osteogenesis imperfecta: - Mild to moderate bone fragility. Sclera pale in early life, but fades in later life. Fractures present in 50 % case – frequency of fractures decreases after puberty. Some patients may have dentinogenesis imperfecta, some may not. Slide 22: HISTOLOGICAL FEATURES: - Anomaly due to abnormal collagen synthesis by abnormal osteoblasts. Mass of cortical and cancellous bone greatly reduced. Osteoblasts present but bone matrix synthesis is reduced. Bone architecture remains immature throughout life. CLEIDOCRANIAL DYSPLASIA : CLEIDOCRANIAL DYSPLASIA Bone defects primarily involve skull and clavicle – defects seen in other bone also. Inherited as autosomal dominant trait, but almost 40% cases show spontaneous mutation. Slide 24: CLINICAL FEATURES: - Age incidence: Children Sex incidence: Nil Site predilection: Skull, clavicles and jaw bones. Slide 25: Signs & symptoms: Short height with large heads showing pronounced frontal and parietal bossing. Nose is broad with depressed nasal bridge. Shoulders narrow and droop excessively. Patients show unusual mobility of shoulders due to absence / hypoplasia of clavicles. Slide 26: Oral manifestations: Narrow, high arched palate. Increased prevalence of cleft palate. Prolonged retention of deciduous teeth and delay / complete failure of eruption of permanent teeth. OPG and dental radiographs show multiple impacted and supernumerary teeth. OSTEOPETROSIS(Albers - Schönberg Disease, Marble bone disease) : OSTEOPETROSIS(Albers - Schönberg Disease, Marble bone disease) Rare hereditary bone disorder characterized by increase in bone density due to defect in bone remodeling caused by failure of normal osteoclast function. Two clinical types – infantile and adult osteopetrosis. Slide 28: PATHOGENESIS: - Osteoclasts fail to function normally. As a result, bone remodeling is affected. Defective bone resorption combined with continued bone deposition results in thickening of cortical bone and sclerosis of cancellous bone. Slide 29: I. INFANTILE OSTEOPETROSIS CLINCAL FEATURES: - Autosomal recessive trait. Diffusely sclerotic skeleton, marrow failure and signs of cranial nerve compression present. Initial signs – normocytic normochromic anemia and hepatosplenomegaly. Increased susceptibility to infections due to granulocytopenia. Slide 30: Oral manifestations: Facial deformity leading to hypertelorism, snub nose, frontal bossing etc. Delayed tooth eruption and osteomyelitis of jaws. Sclerosis of skull bones leads to narrowing of foramina which causes compression of various cranial nerves – blindness, deafness, facial paralysis etc Slide 31: RADIOGRAPHIC FEATURES: - Wide spread increase in bone density. Distinction between cortical and cancellous bone is lost. Dental X rays – difficult to distinguish roots. Slide 32: II. ADULT OSTEOPETROSIS CLINICAL FEATURES: - Discovered late in life – milder symptoms. Autosomal dominant trait. About 40% cases are asymptomatic. Axial skeleton shows sclerosis, while long bones show little or no defects. Slide 33: HISTOLOGICAL FEATURES: Several types of abnormal endosteal formation. - Tortuous lamellar trabeculae. - Globular amorphous bone. - Osteophytic bone formation. IDIOPATHIC DISEASES : IDIOPATHIC DISEASES PAGET’S DISEASE OF BONE(Osteitis deformans) : PAGET’S DISEASE OF BONE(Osteitis deformans) Characterized by abnormal resorption and deposition of bone, resulting in distortion and weakening of bone. ETIOLOGY: - Unknown, but predisposing factors could be – inflammatory, genetic, endocrine factors or a slow virus infection. Slide 36: CLINICAL FEATURES: - Age incidence: Middle aged individuals Sex incidence: Male to female ratio is 2:1 Site predilection: Bones of skull, lumbar vertebrae pelvis, femur and tibia. Slide 37: Signs & symptoms: Severe bone pain and limitation of movement, especially of joints. Affected bones – thickened, enlarged and weak. Weight bearing joints become bowed. Skull involvement – increase in head circumference. Slide 38: Signs & symptoms: Maxilla affected more than mandible. Maxilla – enlargement of middle third of face (leontiasis ossea) Nasal obstruction, obliterated sinuses and deviated septum also occur. In dentulous patients – spacing of teeth. Mandible involved rarely – may cause prognathism. Slide 39: RADIOGRAPHIC FEATURES: Early stage (lytic) -radiolucency and alteration of trabecular pattern. Late stage (osteoblastic) – patchy areas of sclerotic bone is formed, called “cotton wool” appearance. Slide 40: Identify the disease.. Write its predisposing factors. Slide 41: Dental radiographs also show the classical cotton wool appearance. Extensive hypercementosis can be noted. Slide 42: DIFFERENTIAL DIAGNOSIS: - Acromegaly. Florid cemento- osseous dysplasia. Sclerosing osteomyelitis (diffuse type). Osteosarcoma. Adult osteopetrosis Slide 43: LABORATORYFINDINGS: - Abnormally elevated serum alkaline phosphatase level upto 250 Bodansky units (normal – 30 to 40). But normal calcium and phosphorous levels). Increased urinary calcium and hydroxyproline levels. Slide 44: HISTOLOGICAL FEATURES: - Alternating bone resorption and deposition seen. Thus osteoclastic resorption seen surrounding the trabeculae. Simultaneously, osteoblastic activity also seen with formation of osteoid rims around trabeculae. Surrounding stroma is highly fibrovascular. Slide 45: A characteristic feature is presence of basophilic reversal lines in the bones. This indicates junction between the alternating resorptive and formative phases of bone. It gives a “jigsaw puzzle” or “mosaic” appearance of the bone. Slide 46: DENTAL COMPLICATIONS: - Hypercementosis and ankylosis of teeth – difficulty in extraction. Increased bleeding, dry socket and osteomyelitis may occur after extraction. Edentulous patients change dentures from time to time due to enlargement of alveolar ridges. Pathological fracture. LANGERHANS CELL DISEASE : LANGERHANS CELL DISEASE It is an idiopathic disease characterized by proliferation of histiocyte like cells (Langerhan's cells), that are accompanied by varying numbers of eosinophils, lymphocytes, plasma cells & multinucleated giant cell. Believed to be a non neoplastic process. Langerhan’s cells are dendritic, mononuclear cells normally found in epidermis, mucosa , lymph nodes & bone marrow. They are known as antigen presenting cells Slide 48: TYPES: - Eosinophilic granuloma of bone: solitary or multiple bone involvement without systemic organ involvement. Hand-Schüller-Christian disease: chronic disseminated disease involving bones, viscera and skin. Letter-Siwe disease: acute disseminated disease with bone, visceral and skin involvement, occurring mainly in infants. Slide 49: CLINICAL FEATURES: - Age incidence: Predominantly children below 10 years of age. Sex incidence: Definite male predilection. Site predilection: Bones - Skull, ribs, vertebrae, femur and mandible most frequently. Oral – gingiva and lips most commonly. Slide 50: Signs & symptoms: Involved bones manifest dull pain and tenderness. Visceral involvement results in decreased or failure of affected organ. Slide 51: Jaw bone involvement results in loosening of teeth which resembles aggressive periodontitis. Oral mucosa may show ulcerative / proliferative masses on gingiva. Slide 52: RADIOGRAPHIC FEATURES: - Multiple, well / poorly defined punched out radiolucent areas seen. Extensive alveolar bone loss occurs, causing the teeth to appear as if they are “floating in air”. Slide 53: DIFFERENTIAL DIAGNOSIS: - Other lesions with multifocal, multilocular radiolucency are - Cemento-osseous dysplasia - Hyperparathyroidism - Cherubism - Multiple myeloma Slide 54: HISTOLOGICAL FEATURES: - Lesion shows diffuse infiltration of pale staining, mononuclear cells containing ill defined cell borders and vesicular nuclei. Darker staining eosinophils, plasma cells and lymphocytes and multinucleated giant cells also seen. Electron microscopy shows rod / racquet shaped characteristic birbeck granules within cytoplasm of Langerhan's cells. Slide 55: Immunohistochemical studies are needed to confirm the diagnosis as these cells cannot be distinguished from normal histiocytes. Langerhan’s cells stain positively for S-100 protein. REACTIVE LESIONS : REACTIVE LESIONS CENTRAL GIANT CELL GRANULOMA : CENTRAL GIANT CELL GRANULOMA Considered to be a non neoplastic lesion. Can be grouped under two types – Non aggressive type = slow growth, no symptoms Aggressive type = pain, rapid growth seen. Slide 58: CLINICAL FEATURES: - Age incidence: 60% cases occur below 20 years of age. Sex incidence: Predominantly females. Site predilection: 70% cases occur in mandible. Most lesions occur in anterior portions of jaws. Slide 59: Signs & symptoms: Mostly asymptomatic and diagnosed only during routine radiographic examination. Manifest usually as painless expansion of affected bone. Some aggressive cases may manifest with pain, paresthesia and perforation of cortical plate Slide 60: RADIOGRAPHIC FEATURES: - Present as well defined, unilocular / multilocular radiolucent defects, but the margins are usually non sclerotic. Lesions may vary in size from small unilocular radiolucencies to large multilocular radiolucencies. Slide 61: DIFFERENTIAL DIAGNOSIS: - Small unilocular lesions can be confused radiographically with periapical cyst / granuloma. Large multilocular lesions should be differentiated from other multilocular radiolucencies like– ameloblastoma, aneurysmal bone cyst, Pindborg tumor etc Slide 62: HISTOLOGICAL FEATURES: - Few to large number of small / large multinucleated giant cells seen in a background of ovoid / spindle shaped mesenchymal cells. Giant cells believed to represent osteoclasts. Foci of osteoid and newly formed bone may also be seen. Areas of hemorrhage and hemosiderin deposition are common. ANEURYSMAL BONE CYST : ANEURYSMAL BONE CYST Primarily seen in long bones or vertebrae. Cause and pathogenesis are not yet clear. Controversy – whether it arises de novo or occurs as a result of some “vascular accident” in a pre-existing lesion. Slide 64: CLINICAL FEATURES: - Age incidence: First 3 decades. Sex incidence: Mainly females. Site predilection: molar regions of mandible & maxilla. Slide 65: Signs & symptoms: Hard, rapidly growing swelling which can cause malocclusion. Pain is often present. If lesion perforates cortical plates, can cause “egg shell crackling”. Slide 66: RADIOLOGICAL FEATURES: - Classically seen as a unilocular, ovoid / fusiform radiolucency which balloons the cortical plates. Teeth displacement and root resorption also observed. Slide 67: HISTOLOGICAL FEATURES: Cyst cavity shows many capillaries and blood filled spaces, separated by delicate loose CT. Blood filled spaces are not lined by endothelium. Many small multinucleated giant cells and trabeculae of osteoid / woven bone. Many times features of fibrous dysplasia, Ossifying fibroma also seen SIMPLE BONE CYST(Solitary / Traumatic / Hemorrhagic bone cyst} : SIMPLE BONE CYST(Solitary / Traumatic / Hemorrhagic bone cyst} Commonly seen in mandible, rare in maxilla. Identical to solitary bone cyst of humerus in children and adolescents. Slide 69: PATHOGENESIS: - None of the theories are certain about exact cause. First theory – cyst may follow trauma to bone which causes intra medullary hemorrhage which fails to organize. This clot subsequently liquefies - CYST. Recent theory – osteogenic cells fail to differentiate locally and thus instead of bone, the undifferentiated cells form synovial tissue. Slide 70: CLINICAL FEATURES: - Age incidence: Young individuals Sex incidence: Equal Site predilection: Body and symphysis of mandible. Slide 71: Signs & symptoms: Asymptomatic. Rarely, swelling and pain may be seen. Half of all patients give a history of trauma to the area. Slide 72: RADIOGRAPHICAL FEATURES: - Appears as a radiolucency with irregular but well defined edges and slight cortication. When many teeth involved – radiolucency scallops between roots. Teeth involved in lesion – usually vital, no root resorption seen. Slide 73: DIFFERENTIAL DIAGNOSIS: - Odontogenic keratocyst Central giant cell granuloma Periapical cemento-osseous dysplasia (early stage) Ossifying fibroma (early stage) Unicystic ameloblastoma. Slide 74: HISTOLOGICAL FEATURES:- Wall shows loose fibrovascular CT. Hemorrhage and hemosiderin pigment usually present. Multinucleated giant cells scattered within the CT. Adjacent bone shows osteoclastic resorption on inner surface. FIBRO – OSSEOUS LESIONS : FIBRO – OSSEOUS LESIONS Slide 76: Fibro-osseous lesions are a diverse group of lesions characterized by replacement of normal bone by a fibrous tissue containing a newly formed, mineralized product. These lesions include developmental, reactive and even neoplastic lesions. Histologic features can be very similar in lesions of different etiology and biological behavior. Clinical, pathological and radiographic correlation is required to establish a specific diagnosis. Slide 77: CLASSIFICATION: - (i) Non-neoplastic lesions - a. Fibrous dysplasia b. Cemento-osseous dysplasia → Periapical cement-osseous dysplasia → Focal cemento-osseous dysplasia → Florid cemento-osseous dysplasia (ii) Neoplasms – - Ossifying fibroma FIBROUS DYSPLASIA : FIBROUS DYSPLASIA Condition in which normal medullary bone is gradually replaced by an abnormal fibrous connective tissue proliferation. This mesenchymal tissue contains varying amounts of osteoid that presumably arises through metaplasia. The resultant fibro-osseous tissue is poorly formed and structurally inadequate. ▪ The condition tends to stabilize and stops growing as skeletal maturity is reached. Slide 79: ETIOLOGY: - Hamartomatous Abnormal reaction of bone to a localized traumatic episode. Endocrine disturbance: the recent description of presence of estrogen receptors in osteogenic cells of a patient with FD suggests that this process may reflect a defect in the regulation of these receptors and consequently of cellular activity. Slide 80: TYPES OF FIBROUS DYSPLASIA: - Monostotic: Fibrous dysplasia (FD) limited to one single bone. Accounts for 80% – 85% of all cases. Polyostotic: FD affects several bones. (a) Jaffe type – severe FD with almost entire skeleton involved. (b) McCune-Albright syndrome – along with polyostotic FD, multiple cutaneous pigmentations and hyperfunction of one or more endocrine glands. Slide 81: MONOSTOTIC FIBROUS DYSPLASIA CLINICAL FEATURES: - Age incidence: 1st or 2nd decade of life. Sex incidence: equal Site predilection: Maxilla involved more than mandible. Maxillary lesions often involve adjacent bones like zygoma, sphenoid etc (called Craniofacial FD). Slide 82: Signs & symptoms: Affected bone / bones show a painless, gradually enlarging swelling. Teeth within affected jaws remain firm but may be displaced by the mass. Slide 83: RADIOGRAPHIC FEATURES: - Early stages – mixed radiopaque-radiolucent appearance. Later stages show a characteristic “ground glass / orange peel” appearance of affected bones. Lesions not well defined and blend into adjacent bone – limits of lesion cannot be defined. Slide 84: DIFFERENTIAL DIAGNOSIS: - Clinically, FD must be differentiated from Ossifying fibroma Paget’s disease. Though, its radiographic appearance is typical, it must be distinguished from Hyperparathyroidism. Paget’s disease (early stage). Slide 85: POLYOSTOTIC FIBROUS DYSPLASIA CLINICAL FEATURES: - Age incidence: 1st decade of life or earlier. Sex incidence: equal. Site predilection: Can affect any bone in skeleton, but primarily the skull bones and long bones of skeleton. Slide 86: Signs & symptoms: - Even though skull and jaws commonly affected, symptoms are mostly related to involvement of long bones – pain, pathological fractures etc. Patients with McCune-Albright syndrome have café-au-lait (coffee with milk) pigmentation. Typically, margins of the spots are irregular, unlike those of neurofibromatosis, where the spots have smooth borders Slide 87: HISTOLOGICAL FEATURES: - Lesion shows typical irregular, Chinese character shaped trabeculae of immature woven bone in a cellular, vascular stroma. These trabeculae believed to arise due to metaplasia and are not bordered by osteoblasts. The surrounding stroma is highly cellular and vascular. CEMENTO-OSSEOUS DYSPLASIAS : CEMENTO-OSSEOUS DYSPLASIAS Commonest type of fibro-osseous lesions in head and neck region. Believed to represent some form of reactive process. Histopathological features similar to FD and ossifying fibroma. Slide 89: TYPES OF CEMENTO-OSSEOUS DYSPLASIAS: - Based on their clinical and radiological features, grouped into Periapical cemento-osseous dysplasia Focal cemento-osseous dysplasia Florid cemento-osseous dysplasia Slide 90: PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA CLINICAL FEATURES: - Age incidence: 30 – 50 years Sex incidence: marked female predilection (female : male ratio is 14 : 1) Racial predilection: Predominantly blacks Site predilection: predominantly mandibular anteriors. Slide 91: Signs & symptoms: Can occur either as solitary lesion, but mostly as multiple lesions. Almost always asymptomatic. Teeth associated with the lesions are always vital. Slide 92: RADIOGRAPHIC FEATURES: - (a) Early stage: well circumscribed radiolucency involving apices of vital teeth (cannot be distinguished from periapical granuloma / cyst) Slide 93: (b) Intermediate stage: as mineralized areas begin to appear in the lesion, mixed radiopaque radiolucent appearance seen. Slide 94: (c) Late stage: the entire lesion becomes radiopaque surrounded by a narrow radiolucent rim. Slide 95: FOCAL CEMENTO-OSSEOUS DYSPLASIA Benign cemento-osseous lesion. Features intermediate between those of periapical and florid cemento-osseous dysplasia. Believed to be the commonest fibro-osseous lesion. Slide 96: CLINICAL FEATURES: - Age incidence: 4th and 5th decades. Sex incidence: 80 % cases in females. Racial predilection: more in whites. Site predilection: mostly posterior mandible. Signs & symptoms: asymptomatic, detected during routine radiographic examination. Slide 97: RADIOGRAPHIC FEATURES: - Lesion may be completely radiolucent / radiopaque. Most commonly, it is mixed radiopaque-radiolucent. Borders are usually irregular. Slide 98: FLORID CEMENTO-OSSEOUS DYSPLASIA Widespread disease affecting greater area of jaw bones. Secondary infections commonly occur (low grade osteomyelitis) due to exposure of abnormal mineralized material to oral cavity. Slide 99: CLINICAL FEATURES: - Age incidence: 2nd and 3rd decades. Sex incidence: predominantly females. Racial predilection: 90% cases in black women. Site predilection: Mostly bilaterally symmetrical Either jaw may be involved. Slide 100: Signs & symptoms: Usually asymptomatic. Patients may complain of dull pain. In some cases, yellowish, avascular bone like material may be seen exposed to oral cavity. Affected jaw bone may show expansion. Slide 101: RADIOGRAPHIC FEATURES: - Lesions are well defined and radiopaque, often mixed with areas of less well defined mixed radiopaque-radiolucent regions. In some cases, single / multiple simple bone cysts may be associated with this disease. Slide 102: DIFFERENTIAL DIAGNOSIS: - In early stages when the lesion is radiolucent, Nevoid basal cell carcinoma syndrome. Cherubism Multiple myeloma Brown’s tumor of hyperparathyroidism. In later stages when lesion is mixed, Odontoma Ossifying fibroma Ameloblastic fibro-odontoma COC CEOT Slide 103: HISTOLOGICAL FEATURES: - In early stages, lesion shows fibroblastic proliferation which may contain small areas of osteoid formation. No evidence of inflammation. Slide 104: In later stages, the lesion shows increasing deposition of bone or cementum like material. In the final stages, the entire lesion may be composed of dense mineralized tissue. OSSIFYING FIBROMA(Cementifying fibroma / Cemento-ossifying fibroma) : OSSIFYING FIBROMA(Cementifying fibroma / Cemento-ossifying fibroma) Ossifying fibroma (OF) is a well circumscribed, sometimes encapsulated neoplasm composed of fibrous tissue containing varying amounts of calcified material. This calcified material may be bone, cementum or a mixture of both. Slide 106: CLINICAL FEATURES: - Age incidence: 3rd and 4th decades. Sex incidence: Female to male ratio in 5 : 1 Site predilection: Mandible involved more frequently than maxilla. Within mandible, premolar – molar area is the commonest site affected. Slide 107: Signs & symptoms: Small lesions are asymptomatic and detected only during routine radiographic examination. Larger lesions may cause painless expansion of involved bone. Expansion of bone can cause facial asymmetry. Pain and paresthesia are very rarely noted. Slide 108: RADIOGRAPHIC FEATURES: Most often lesions are well defined, unilocular. Some lesions may be mixed radiopaque-radiolucent depending on the amount of calcified material present in the tumor. Large lesion may produce root divergence and root resorption. Slide 109: DIFFERENTIAL DIAGNOSIS: - Fibrous dysplasia Osteoblastoma and osteoid osteoma cementoblastoma Focal sclerosing osteomyelitis. Cemento-osseous dysplasia. Slide 110: HISTOLOGICAL FEATURES: Most tumors are well circumscribed masses composed of fibrous tissue and containing calcified material. The calcified material may be in the form of irregular trabeculae of osteoid or basophilic, globular calcifications resembling cementum. Many times both are present in the same lesion. JUVENILE OSSIFYING FIBROMA : JUVENILE OSSIFYING FIBROMA Uncommon lesion of bone. Differentiated from ossifying fibroma on the basis of age incidence, site predilection and clinical behavior. However, histologically the distinction from OF is not so clear. Two patterns recognized – trabecular and psammomatoid. Slide 112: CLINICAL FEATURES: - Age incidence: Patients younger than 15 years of age. Sex incidence: Equal. Site predilection: Most commonly involves orbital and frontal bones. Maxilla is involved more commonly. Slide 113: Signs & symptoms: Most tumors show rapid growth. In such cases, pain and paresthesia may be noted. Cortical expansion and facial asymmetry is seen with jaw lesions. Orbital and sinus involvement may cause exophthalmus, proptosis and nasal obstruction. Slide 114: RADIOGRAPHIC FEATURES: - Can be radiolucent or mixed radiopaque-radiolucent depending on amount of calcified material present within the tumor. Lesion may be well demarcated or may show invasion into surrounding bone. Slide 115: HISTOLOGICAL FEATURES: - 1. TRABECULAR J.O.F: Both patterns of JOF well circumscribed but not encapsulated. Tumor composed of fibrocellular CT, areas of hemorrhage and occasional multinucleated giant cells. Mineralized component shows irregular strands of osteoid lined by plump osteoblasts. Slide 116: 2. PSAMMOMATOID JOF: The stroma is similar to trabecular JOF. The mineralized material is composed of concentric, lamellated and spherical ossicles. These ossicles vary in size and typically have basophilic centers with eosinophilic osteoid rims. INFECTIOUS DISEASES : INFECTIOUS DISEASES OSTEOMYELITIS : OSTEOMYELITIS Refers to acute / chronic inflammatory process in medullary spaces or cortical surfaces of bones. Various patterns recognized like focal and diffuse sclerosing osteomyelitis, proliferative periostitis etc. Slide 119: TYPES OF OSTEOMYELITIS: - Acute osteomyelitis Chronic osteomyelitis Diffuse sclerosing osteomyelitis Condensing osteitis (Focal sclerosing osteomyelitis) Osteomyelitis with proliferative periostitis (Garre’s osteomyelitis). Alveolar osteitis (Dry socket) Slide 120: PREDISPOSING FACTORS: - After odontogenic infections Trauma to jaws Presence of ANUG Chronic systemic diseases Immunocompromised states Tobacco and alcohol abuse Diabetes mellitus Exanthematous fevers Malignancy Malnutrition ACUTE OSTEOMYELITIS : ACUTE OSTEOMYELITIS Acute osteomyelitis occurs when acute inflammation spreads through medullary spaces of bone. CLINICAL FEATURES: - Age incidence: Any age Sex incidence: Strong male predilection Site predilection: Mostly in mandible. Maxilla involved primarily in children. Slide 122: Signs & symptoms: Fever, leukocytosis, lymphadenopathy and soft tissue swelling of affected area. X-rays can show an ill defined radiolucency. Occasionally, fragments of necrotic bone can be seen separating from surrounding normal bone – Sequestrum. If sequestrum is surrounded by vital bone – Involucrum. Slide 123: HISTOLOGICAL FEATURES: - Biopsy specimen usually contains necrotic bone, showing loss of osteocytes from lacunae and bacterial colonization. Bone periphery shows necrotic debris and infiltration with PMNL’s. Specimen is diagnosed as sequestrum unless there is good clinico-pathologic correlation. CHRONIC OSTEOMYELITIS : CHRONIC OSTEOMYELITIS It can arise either de novo from the onset or as a continuation of acute osteomyelitis, if it is not resolved quickly. CLINICAL FEATURES: - Age incidence: Any age Sex incidence: Strong male predilection Site predilection: Mostly in mandible. Slide 125: Signs & symptoms: Pain, swelling, purulent discharge, sinus formation, sequestrum formation, tooth loss. Frequent acute exacerbations may occur if infection continues for a long time. X-rays reveal ill defined, moth eaten radiolucency often showing a central radiopacity (sequestrum). Slide 126: HISTOLOGICAL FEATURES: Biopsy material contains significant soft tissue component consisting of chronically inflamed fibrous CT filling intertrabecular areas of bone. Scattered areas of sequestrum may also be noted. DIFFUSE SCLEROSING OSTEOMYELITIS : DIFFUSE SCLEROSING OSTEOMYELITIS Characterized by pain, inflammation, varying degrees of periosteal hyperplasia, sclerosis and radiolucency of affected bone. Can be confused clinically and radiologically with certain other intrabony pathoses like florid cemento-osseous dysplasia or Paget's disease of bone etc. Slide 128: CLINICAL FEATURES: - Age incidence: Almost exclusively in adults. Sex incidence: Nil Site predilection: Primarily in mandible Signs & symptoms: Pain and swelling are uncommon. To make a definitive diagnosis of diffuse sclerosing osteomyelitis, microbiological cultures must be positive. Slide 129: RADIOGRAPHIC FEATURES: - Increased radiopacity around sites of chronic inflammation like periodontitis, pericoronitis, periapical pathology etc. Sclerosis occurs more in alveolar crest regions of tooth bearing areas. Slide 130: HISTOLOGICAL FEATURES: - Sclerosis and remodeling of bone. Significant inflammation of bone is not seen even though sclerosis occurs adjacent to inflammation. Necrosis of sclerotic bone secondary to inflammation may occur. In this case, necrotic bone separates and is surrounded by granulation tissue FOCAL SCLEROSING OSTEOMYELITIS(Condensing osteitis) : FOCAL SCLEROSING OSTEOMYELITIS(Condensing osteitis) This refers to a focal area of bone sclerosis associated with apices of pulpally involved (caries, deep restorations or pulp necrosis) teeth. To be diagnosed as condensing osteitis, association with inflammation is essential, as it resembles several other intrabony pathoses. Slide 132: CLINICAL FEATURES: - Occurs mostly in children and young adults. Mostly occurs in mandibular premolar / molar area, associated with pulpitis / pulp necrosis. Localized, uniform zone of increased radiopacity seen adjacent to tooth apex. No swelling / cortical expansion noted clinically. Slide 133: DIFFERENTIAL DIAGNOSIS: - This lesion must be distinguished from Focal cemento osseous dysplasia – it shows a radiolucent border. Idiopathic osteosclerosis – here, the lesion is separated from the tooth apex. OSTEOMYELITIS WITH PROLIFERATIVE PERIOSTITIS : OSTEOMYELITIS WITH PROLIFERATIVE PERIOSTITIS Also called Periostitis ossificans or Garrѐ’s Osteomyelitis. It is a type of osteomyelitis associated with periosteal bone formation. Slide 135: CLINICAL FEATURES: - Age incidence: Children & young adults Sex incidence: Nil Site predilection: Mostly in premolar / molar regions of mandible. Slide 136: Signs & symptoms: Swelling may be noted on lower border of mandible. Pain may / may not be present. Radiographs demonstrate radiopaque laminations roughly parallel to each other and the underlying cortical surface (onion skin appearance). Slide 137: HISTOLOGICAL FEATURES: Shows parallel rows of highly cellular, woven bone in which the individual trabeculae are oriented perpendicular to surface. Sometimes, trabeculae are interconnected or they may be scattered, resembling fibrous dysplasia. In between trabeculae, fibrous CT is relatively non inflamed. ALVEOLAR OSTEITIS(Dry socket / Fibrinolytic alveolitis) : ALVEOLAR OSTEITIS(Dry socket / Fibrinolytic alveolitis) Sometimes, the blood clot at the extraction site fails to organize which eventually leads to delayed healing and causes a condition called “Dry socket”. Research shows it is due to transformation of plasminogen to plasmin with resultant lysis of fibrin and formation of kinin (pain mediators). Slide 139: PREDISPOSING FACTORS: - Local trauma Estrogens Bacterial toxins Inadequate irrigation of surgery site Tobacco abuse. Slide 140: CLINICAL FEATURES: - Age incidence: Between 20 – 40 years Sex incidence: Nil Site predilection: Posterior mandibular teeth, especially impacted third molars. Slide 141: Signs & symptoms: Affected extraction site filled with a dirty gray clot, which is lost, leaving behind a bare, bony socket (Dry socket). Diagnosis is confirmed by probing of socket which shows an exposed and extremely sensitive bone. Severe pain, foul smell and lymphadenopathy develop within 3 – 4 days of extraction. NEOPLASTIC DISORDERS : NEOPLASTIC DISORDERS OSTEOMA : OSTEOMA Benign tumors composed of mature compact / cancellous bone. Most commonly occur in craniofacial skeleton – rare in other parts of body. Palatal and mandibular tori are not considered as osteomas although they are histologically identical. Slide 144: TYPES OF OSTEOMA: - I. Depending on location: - Periosteal - Endosteal II. Depending on type of bone: - Compact - Cancellous Slide 145: CLINICAL FEATURES: - Age incidence: Young adults. Sex incidence: Nil Site predilection: Mandible affected more commonly than maxilla. In mandible – body / condyle. Body of mandible – posterior to premolars on lingual surface. Slide 146: Signs & symptoms: Periosteal – slowly growing polypoid / sessile mass, usually solitary. Endosteal – usually seen in condyles, cause progressive shift in patient’s occlusion towards unaffected side. Other signs include facial pain swelling and limited mouth opening. Slide 147: RADIOGRAPHIC FEATURES: - Endosteal osteomas appear as radiopaque sclerotic masses. Periosteal osteomas may appear as uniform ‘opaque mass or sclerotic periphery with central trabecular pattern. Slide 148: DIFFERENTIAL DIAGNOSIS: - Exostoses Osteoblastoma and osteoid osteoma: Odontomes Focal sclerosing osteomyelitis. . : . HISTOLOGICAL FEATURES: - 1. Compact Osteoma: Normal appearing mature compact bone showing minimal marrow tissue. 2. Cancellous osteoma: Trabeculae of bone and fibrofatty marrow. Significant osteoblastic activity may be seen Slide 150: GARDNER SYNDROME Multiple osteomas Multiple intestinal polypoid lesions Multiple epidermoid and dermoid cysts Multiple supernumerary teeth OSTEOSARCOMA(Osteogenic sarcoma) : OSTEOSARCOMA(Osteogenic sarcoma) Malignancy of mesenchymal cells that have the ability to produce osteoid or immature bone. Commonest malignancy arising within the bone along with hematopoietic neoplasms. Majority arise from within the bone (intramedullary), some may be peripheral (juxtacortical) Slide 153: CLINICAL FEATURES: - Age incidence: 3rd and 4th decades. Sex incidence: Commoner in males. Site predilection: Long bones and U / L jaws. Slide 154: Signs & symptoms: Swelling and pain - commonest symptoms. Loosening of teeth, paresthesia and nasal obstruction (in case of maxillary tumors) may also be noted. Slide 155: RADIOGRAPHIC FEATURES: - Radiographic features vary from densely sclerotic Slide 156: Mixed radiopacity – radiolucency (mottled) Slide 157: To completely radiopaque Periphery of lesions usually indistinct and ill defined. Slide 158: The characteristic “sunburst” appearance can be noted in about 25% of jaw tumors. Produced by osteophytic bone production. Slide 159: DIFFERENTIAL DIAGNOSIS: - - Osteoblastoma - Fibrous dysplasia - Ossifying fibroma Slide 160: HISTOLOGICAL FEATURES: - Considerable variation seen. Essentially – osteoid production by malignant mesenchymal cells. In addition to osteoid, chondroid and fibrous material also seen many times. Tumor cells may vary from spindle shaped to highly pleomorphic types. Slide 161: Osteosarcomas can be classified on the basis of relative amounts of chondroid / osteoid / fibers produced by tumor into: 1. Chondroblastic Slide 162: 2. Fibroblastic Slide 163: 3. Osteoblastic CHONDROMA : CHONDROMA Benign tumors composed of mature hyaline cartilage. Common bone tumor, occurring mostly in short bones of hands and feet. Occur very rarely in jaw bones. Jaw tumors occur usually in anterior maxilla of adult patients. Slide 165: HISTOLOGICAL FEATURES: - Very difficult to differentiate between a benign chondroma and well differentiated, low grade chondrosarcoma. Chondroma composed of mature hyaline cartilage. However, a diagnosis of chondroma for jaw lesion is rarely given as most of them are malignant. CHONDROSARCOMA : CHONDROSARCOMA Malignant tumor characterized by cartilage formation, but not bone, by the tumor cells. They comprise about 10% of all primary bone tumors of skeleton, but occur very rarely in the jaws. Slide 167: CLINICAL FEATURES: - Age incidence: Occurs in a wide age range. Average age incidence is 3rd decade. Sex incidence: Slightly more in males. Site predilection: Involves maxilla and mandible with equal frequency. Slide 168: Signs & symptoms: Manifests usually as a painless swelling. There may be separation and loosening of teeth also. Pain is not usually a feature of this tumor, as in case of osteosarcoma. Maxillary tumors – nasal obstruction or epitaxis. Slide 169: RADIOGRAPHIC FEATURES: Usually seen as poorly defined radiolucency with variable amounts of radiopaque foci (caused by calcification of cartilage matrix). If tumor penetrates cortex – sunburst appearance. Slide 170: DIFFERENTIAL DIAGNOSIS: - Osteosarcoma (especially if tumor shows sunburst appearance). Osteomyelitis. Periapical granuloma. Ewing’s sarcoma. Primary intraosseous carcinomas like Mucoepidermoid carcinoma, Ameloblastic carcinoma etc. Slide 171: HISTOLOGICAL FEATURES: Composed of cartilage showing varying degrees of maturation and cellularity. Most cases – typical lacuna formation with chondroid matrix. Lobular growth pattern seen with centre of lobule showing greatest maturation and periphery showing immature cartilage along with a stroma of round / spindle cells. EWING’S SARCOMA : EWING’S SARCOMA Primary malignant tumor of bone. Histogenesis is uncertain. Comprises 6 % – 10 % of all primary bone tumors. Earlier believed to arise from endothelial cells, hematopoietic cells or undifferentiated mesenchymal cells. Now – possibly neuroectodermal origin. Slide 173: CLINICAL FEATURES: - Age incidence: Children & adolescents. Sex incidence: more than 60% cases occur in males. Racial incidence: Predominantly in whites. Site predilection: Primarily affects femur and pelvic bones. Jaw tumors very rare. Slide 174: Signs & symptoms: Pain and swelling are the commonest manifestations. Pain is usually intermittent and can be dull or severe. Other signs include paresthesia and tooth mobility. General signs – fever & elevated ESR (can be mistaken for osteomyelitis). Slide 175: RADIOGRAPHIC FEATURES: - Seen as irregular, radiolucent lesion with poorly defined margins. Cortical destruction or expansion is not usually seen. Slide 176: HISTOLOGICAL FEATURES: - Composed of small round cells with indistinct cell outlines but well defined nuclear boundary. Tumor cells proliferate in sheets without any pattern. Large areas of necrosis and hemorrhage also seen. Diagnosis difficult, as it is similar to lymphomas, small cell osteosarcoma, embryonal rhabdomyosarcoma etc. METASTATIC TUMORS TO JAWS : METASTATIC TUMORS TO JAWS Slide 178: Definition: Metastasis is the transfer of disease from one organ or part to another organ or part not directly connected with it. Pathogenesis: Disease can spread to other regions through - Hematogenous spread - Lymphatic spread - Perineural invasion Slide 179: Types of metastases to jaws: - Adenocarcinomas - Carcinomas - Sarcomas Sites of primary tumors: - Breast - Prostrate - Kidney - Lungs - Thyroid Slide 180: CLINICAL FEATURES: - Age incidence: Older age persons Sex incidence: Nil Site predilection: More than 80% cases of jaw metastasis occurs in mandible. Signs & symptoms: Pain, swelling, tooth mobility. Inferior alveolar nerve involvement may cause paresthesia and anesthesia. Occasionally, patients are asymptomatic and diagnosis occurs only after microscopic examination after the lesion is noted on a radiograph. Slide 181: RADIOGRAPHIC FEATURES: - Metastatic deposits in the jaws appear as radiolucent defects. These defects may be either poorly defined “moth eaten” radiolucencies or rarely, well circumscribed like a cyst. Some carcinomas, particularly from breast and prostrate may stimulate new bone formation, resulting in a mixed radiopaque – radiolucent lesion. Slide 182: HISTOLOGICAL FEATURES: - Varied presentation. Some times – metastatic deposits well differentiated and closely resemble primary malignancy of specific site like kidney, thyroid etc. Most commonly however, the deposits are poorly differentiated and histological study does not provide any clue to the primary site of tumor. BIBLIOGRAPHY : BIBLIOGRAPHY Shafer WG, Hine MK, Levy BM. A text book of oral pathology. 6th ed. W.B. Saunders Company. Phil, London, Toronto, 2005. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and maxillofacial pathology. 2nd ed. WB Saunders Company. Phil, London, Toronto, 2007. Cawson RA, Odell EW, Porter S. Cawson’s essentials of oral pathology and oral medicine, 7th Ed, Churchill Livingstone, 2002. Regezi JA, Sciubba JJ, Jordan RCK. Oral pathology: Clinical Pathologic Correlations. 4th ed. Saunders Company, 2003. ACKNOWLEDGEMENT : ACKNOWLEDGEMENT All pictures in this presentation are courtesy of authors mentioned in the bibliography and the following authors also: Color Guide Oral Pathology. R A Cawson, F W Odell. 1st ed. Churchill Livingstone, New York. Neville’s color atlas of clinical Oral Pathology, 2nd edition. Slide 185: Thanks for your patience! You do not have the permission to view this presentation. In order to view it, please contact the author of the presentation.