Bone Tumors 2

Bone Tumors 2 Dr. Rima Safadi 28/7/2008

Osteosarcoma 

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Most common primary malignant tumor in jaws Age of onset around 30 May be central, or in relation to periosteum • Juxtacortical have better prognosis than intramedullary

Osteosarcoma 

Presenting signs and symptoms • Swelling • Pain, toothache • Loose or displaced teeth • Bleeding • Paresthesia

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Increased incidence in Paget’s disease

Osteosarcoma 

Radiographic Features • Radiolucent, radiopaque, or mixed • Margins are poorly defined

Osteosarcoma

• “Sun-ray” appearance: Present in only 25% of cases and not unique to osteosarcoma.

Osteosarcoma

Early feature: localized symmetrical widening of periodontal ligament space

Osteosarcoma 

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Microscopic: abnormal osteoblasts and bone Treatment and Prognosis • Radical surgery, radiation therapy, chemotherapy • Mandibular lesions have better prognosis

Chondrosarcoma 

Clinical Features • Chondrosarcoma is more common than chondroma • slower growth rate than other sarcomas of bone • Maxilla>mandible

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Radiographic Features • Radiolucent, radiopaque or mixed • Poorly defined

Chondrosarcoma 

Microscopic Features • Abnormal chondroblasts and cartilage • May be difficult to distinguish from chondroma

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Treatment and Prognosis • Radical surgery • Poorer prognosis than osteosarcoma of jaws

Chondroma

Chondrosarcoma

Questions/Comments??

Multiple Myeloma   

Malignant neoplasm of plasma cells Solitary: plasmacytoma Jaw lesions in both

Multiple Myeloma 

Monoclonal proliferation of plasma cells • Production of a single type of Ig 

Most commonly IgG

• Increased level in serum: paraprotein or M protein

Multiple Myeloma Clinical picture:  50-70 year  Skull, vertebrae, sternum, ribs and pelvic most commonly affected

Multiple Myeloma Diagnosis  Electrophoreses  Bence Johns proteins: light chains in 50% of patients identified in urine  Hypercalcimia  Amyloidosis Treatment: chemotherapy

Multiple Myeloma  

Radiographic features: Sharply demarcated, punched out radiolucent lesions

M spike

Multiple Myeloma  

Microscopic features: Sheets of malignant plasma cells • Binucleation or multinucleation

Questions/Comments??

Langerhans Cell Histiocytosis 

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Langerhans cells: epidermis, lymph nodes, mucosa, bone marrow APC Proliferation of Langerhans cells accompanied by: eosinophils, plasma cells and multinucleated giant cells • Monoclonal…..neoplastic

Langerhans cell histiocytosis 3 clinical forms: 1. Monostotic, solitary eosinophilic granuloma of bone 2. Multifocal: bone and other organs • Craniofacial, orbit, posterior pituitary gland: Hand Schuller Christian syndrome • Skull defects, exophthalmus and diabetes insipidus

Langerhans cell histiocytosis 3. Disseminated histiocytosis, bone skin and viscera (chronic and acute) • Letterer Siewe disease • Fatal, < 2yrs

Langerhans Cell histiocytosis Unifocal and multifocal Skull and jaws <20 yrs Male >female Mandible>maxilla

Langerhans Cell histiocytosis Clinically: loosening of teeth, gingival ulceration, or enlargement  May simulate a periapical lesion  Radiographically: floating in air , punched out radiolucency

Langerhans cell histiocytosis Histopathologically:  Large, pale staining cells resemble histiocytes, round indented nuclei • Variable number of eosinophils

Electron microscope: Birbek granules Immunohistochemistry: surface Ag: CD1-a

Birbek granules

Langerhans Cell Histiocytosis Treatment:  Curettage  Radiotherapy  Intralesional injection with steroid?

Questions/Comments??

Hemangioma of Bone   

Multilocular radiolucency Aspiration: fresh blood Biopsy: cavernous type

Hemangioma

Hemangioma

Central hemangioma

Ossifying Fibroma (cemento-ossifying fibroma)  

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Benign neoplasm, true neoplasm Fibrous tissue, bone, rounded calcified bodies Demarcated with occasional encapsulation

Ossifying Fibroma Clinically:  Swelling, enlarging  Mandible  Sinonasal complex and orbit  Female more?  Radiographically:  RL, RL and RO,

Ossifying Fibroma Histopathology:  Fibrous tissue, well demarcated, trabeculae of bone, osteoblastic rimming  Acellular calcified material  D/D: fibrous dysplasia and cementoosseous dysplasia

Ossifying Fibroma Prognosis: Slowly growing or rapidly growing Juvenile ossifying fibroma: D/D: osteosarcoma 30-60% recurrence rate May be associated with hereditary hyperparathyroidism

Metastatic Tumors 

1% of oral cavity tumors

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Bone metastasis > soft tissue

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Mandible>maxilla

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Carcinoma: breast prostate lung, bronchus, kidney Osteolytic vs osteoblastic (breast, prostate)

Metastatic carcinoma

Metastatic carcinoma

Metastatic disease

Mts