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    Bone Tumors 3 and 4Dr. Rima Safadi

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    Tumors of Bone

    Source: marrow, cartilage, bone,fibrous, vascular

    Benign and malignant

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    Benign tumors

    Osteoma

    Osteoblastoma

    Chondroma

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    Osteoma

    Benign slow growing tumor

    Mature bone

    Mandible>maxilla

    Sub periosteal or central

    Usually solitary

    D/D: dense bone island (centralosteoma), exostosis (subperiostealosteoma) Osteoma: persistent and slowly growing

    Radiographically: well defined

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    Histology: compact (few marrowspaces) or cancellous

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    Osteoma

    Multiple osteomas of the jaws inGardner syndrome

    AD

    Polyposis coli marked tendency formalignant change

    Fibrous tumors Sebacous cysts of the skin

    Multiple impacted and supernumeraryteeth

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    Osteoblastoma

    Histologically andradiographicallyresembling

    cementoblastoma However: not

    related to the rootsof teeth

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    Cementoblastoma

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    Osteosarcoma

    Most common primary malignanttumor in jaws

    Age of onset around 30

    May be central, or in relation toperiosteum

    Juxtacortical have better prognosis thanintramedullary

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    Osteosarcoma

    Presenting signs and symptoms

    Swelling

    Pain, toothache

    Loose or displaced teeth

    Bleeding

    Paresthesia

    Increased incidence in Pagetsdisease

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    Osteosarcoma

    Radiographic Features

    Radiolucent, radiopaque, or mixed

    Margins are poorly defined

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    Osteosarcoma

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    Sun-ray appearance:

    Present in only 25% of cases andnot unique to osteosarcoma.

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    Osteosarcoma

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    Early feature: localizedsymmetrical widening ofperiodontal ligament space

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    Osteosarcoma

    Microscopic: abnormal osteoblastsand bone

    Treatment and Prognosis

    Radical surgery, radiation therapy,chemotherapy

    Mandibular lesions have better

    prognosis

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    Chondrosarcoma

    Clinical Features

    Chondrosarcoma is more common thanchondroma

    slower growth rate than other sarcomas ofbone

    Maxilla (anterior part)>mandible (posteriorpart, condylar process)

    Radiographic Features Radiolucent, radiopaque or mixed

    Poorly defined

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    Chondrosarcoma

    Microscopic Features

    Abnormal chondroblasts and cartilage

    May be difficult to distinguish from

    chondroma

    Treatment and Prognosis

    Radical surgery

    Better prognosis for mandibular lesionsthan maxillary

    Chondroma

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    Chondromabenign tumor, form mature

    cartilage

    Chondrosarcoma

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    Chondrosarcomahigh cellularity, plump cells,

    binucleation

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    Questions/Comments??

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    Multiple Myeloma

    Malignant neoplasm of plasma cells

    Solitary: plasmacytoma

    Jaw lesions in both

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    Multiple Myeloma

    Monoclonal proliferation ofplasma cells

    Production of large amounts ofsingle type of Ig

    Most commonly IgG

    Increased level in serum: paraprotein or M protein

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    Multiple Myeloma

    Clinical picture:

    50-70 year

    Skull, vertebrae, sternum, ribs andpelvic most commonly affected

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    Multiple Myeloma

    Diagnosis Electrophoreses

    Bence Johns proteins: light chains in50% of patients identified in urine

    Hypercalcimia

    Amyloidosis

    Treatment: chemotherapy

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    Multiple Myeloma

    Radiographic features:

    Sharply demarcated, punched outradiolucent lesions

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    M spike

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    Multiple Myeloma

    Microscopic features:

    Sheets of malignant plasma cells

    Binucleation or multinucleation

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    Questions/Comments??

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    Langerhans Cell Histiocytosis

    Langerhans cells: epidermis, lymphnodes, mucosa, bone marrow

    APC

    Proliferation of Langerhans cellsaccompanied by: eosinophils, plasmacells and multinucleated giant cells

    Monoclonal..neoplastic

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    Langerhans cell histiocytosis

    3 clinical forms:

    1. Solitary eosinophilic granuloma ofbone

    2. Multifocal: bone and other organs

    Craniofacial, orbit, posterior pituitarygland: Hand Schuller Christian

    syndrome (triad) Skull defects, exophthalmus and diabetes

    insipidus

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    Langerhans cell histiocytosis

    3. Disseminated histiocytosis, boneskin and viscera (chronic and acute)

    Letterer Siewe disease

    Fatal, < 2yrs of age

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    Langerhans Cell histiocytosis

    Unifocal and multifocal

    Skull and jaws

    female

    Mandible>maxilla

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    Langerhans Cell histiocytosis

    Clinically: loosening of teeth, gingivalulceration, or enlargement

    May simulate a periapical lesion

    Radiographically: floating in air.

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    Langerhans cell histiocytosis

    Histopathologically:

    Large, pale staining cells resemblehistiocytes, round indented nuclei

    Variable number of eosinophils

    Electron microscope: Birbek granules

    Immunohistochemistry: surface Ag:CD1-a

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    Birbek granules

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    Langerhans Cell Histiocytosis

    Treatment:

    Curettage

    Radiotherapy

    Intralesional injection with steroid?

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    Questions/Comments??

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    Hemangioma of Bone

    Multilocular radiolucency

    Aspiration: fresh blood

    Biopsy: cavernous type

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    Hemangioma

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    Hemangioma

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    Central hemangioma

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    Ossifying Fibroma

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    Ossifying Fibroma(cemento-ossifying fibroma)

    Benign neoplasm, true neoplasm

    Fibrous tissue, bone, roundedcalcified bodies

    Demarcated with occasionalencapsulation

    Ossifying Fibroma

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    Ossifying Fibroma

    Clinically:

    Swelling, enlarging

    Mandible

    Sinonasal complex and orbit

    Female more?

    Radiographically: RL, RL and RO,

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    Ossifying Fibroma

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    Ossifying Fibroma

    Histopathology:

    Fibrous tissue, well demarcated,trabeculae of bone, osteoblastic rimming

    Acellular calcified material (resemblingcementum)

    If predominant it is calledpsammomatoid variant (sand-like)

    D/D: fibrous dysplasia and cemento-osseous dysplasia

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    Ossifying Fibroma

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    Ossifying Fibroma

    Prognosis:

    Slowly growing or rapidly growing

    May be associated with hereditaryhyperparathyroidism

    Juvenile ossifying fibroma

    Richly cellula, mitotically active, immature wovenbone

    D/D: osteosarcoma 30-60% recurrence rate

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    Metastatic carcinoma

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    Metastatic carcinoma

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    Metastatic disease

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    Mts

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