Paget`s Disease of Bone and bone tumors1
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Transcript of Paget`s Disease of Bone and bone tumors1
Paget`s Disease of Boneand Bone Tumors 1
Dr. Rima Safadi
27/7/2008
Paget`s Disease of Bone
• Disorganized formation and remodeling of bone unrelated to functional requirements
• Abnormal osteoclastic function
• Etiology:
• Unknown; maybe paramyxovirus infection.– Latency in osteoclast progenitor cells
• Genetic predisposition– Chromosome 18q locus
Paget’s DiseasePathogenesis
3 overlapping phases1. Predominantly osteolytic phase
Increased osteoclastic resorption
2. Active stage of mixed osteolysis and osteogenesis
Abnormal osteoblastic deposition and increased vascularity
3. Sclerotic phaseDense bone and decreased vascularity
Paget’s Disease of BoneClinical Features
• Age: patients over age 40. – More common with increasing age.– 3% overall incidence
• Geographic differences in incidence– More in UK, Australia, North America –
• Gradual onset. Chronic course.
• Involves multiple areas of skeleton– mainly axial skeleton,
more in sacrum– skull and femur– Jaw lesions more
common in maxilla
• May involve single or few bones or disseminated
• Deformity of bones
• Bone pain
• Increased pathologic fracture
Paget’s Disease of BoneClinical Features
• Enlargement of skull and facial bones.
• Narrowing of skull foramina may cause cranial nerve dysfunction
Paget’s DiseaseOral Features
– Enlargement of jaws
– Widening of alveolar ridges.
– Dentures don’t fit– Spaces between
teeth– Facial deformity– Derangement of
occlusion
Widened and flattened palate
Paget’s DiseaseOral Features
When teeth are involved:• Ankylosis• Hypercementosis• Root resorption in
osteolytic phase• Postextraction
hemorrhage in active phase
• More prone to infection in sclerotic phase
Paget’s Disease of BoneRadiographic Features
• Osteoporosis in early phases
• In later stages, patchy osteosclerosis– poorly-defined
radiopaque areas (“cotton wool”)
• In the skull: thickening of the outer plate, loss of distinction between tables
• Teeth: – Loss of lamina duras– Hypercementosis– Ankylosis
Loss of diploe
Paget’s Disease• Microscopic:
– Increased bone turnover – Bone resorbtion and deposition reflects the disease
stages.• The resorbed areas are filled by cellular and vascular fibrous
marrow• Simultaneous osteoclastic and osteoblastic activity• Dense sclerotic bone
– Cementum may also show disorganized remodeling• Hypercementosis and ankylosis
– Osteosarcoma
• Laboratory: – Increased serum alkaline phosphatase– Serum calcium and phosphorus occasionally
increased usually within normal
Numerous reversal lines
Paget’s DiseaseTreatment and Prognosis
• Calcitonin and diphosphonates inhibit osteoclastic resorption.
• Paget’s disease is usually not fatal but has complications.– Increased incidence of malignant neoplasms
(<1% of patients), most commonly osteosarcoma.
Questions/Comments?
Giant Cell Lesions of Bone
• Central giant cell granuloma
• Giant cell tumor of bone
• Giant cell lesions of hyperparathyroidism (“brown tumor”)
• Cherubism
Central Giant Cell GranulomaClinical Features
• Probably reactive and not neoplastic– Reactive to heamodynamic changes: trauma
or heamorrhage
• Most common in young adults
• Female predominance
• Only occurs in the jawsOnly occurs in the jaws
• More common in mandible (~70%)
• Involves anterior part of the jaws
Central Giant Cell Granuloma
• Clinically:– Swelling of bone– Rapid growth or– symptomless
• Non-aggressive lesions: asymptomatic, slow growth, does not perforate cortex, low recurrence
• Aggressive lesions: rapid growth, perforates cortex, higher recurrence
• Most CGCG are non-aggressive
Central Giant Cell GranulomaRadiographic Features
• Well-demarcated or poorly defined radiolucent lesion
• Unilocular or multilocular
• Can cause thinning, expansion or perforation of cortical plate
• Root displacement or resorption.
Central Giant Cell GranulomaMicroscopic Features
• Fibrous connective tissue, multinucleated giant cells, hemorrhage and hemosiderin
1
2
Central Giant Cell GranulomaTreatment and Prognosis
• Simple enucleation and curettage
• Recurrence rate following curettage is about 15-20%
• Long-term prognosis is good.
• Need to exclude hyperparathyroidism
“True” Giant Cell Tumor of Bone
• Giant cell tumors occur in long bones, very rare in Jaws
• more aggressive• higher recurrence rate • may metastasize in 10% of cases• distinct from central giant cell granuloma
and represents a true neoplasm.
Questions/Comments??
Exostoses
Localized bony protuberances, non neoplastic
• Torus palatinus
• Torus mandibularis
• Buccal exostosis
• Palatal exostosis
Torus palatinus• Etiology: genetic versus environmental • Arises at the midline of the hard palate
• Can slowly increase in size• Peak prevalence is in adult- dynamic
Nodular torus Lobular torusSpindle torus and flat torus
Torus mandibularis
• At the lingual aspect• Present above the
mylohyoid line• 90% bilateral• Varies in size• Can correlate with
bruxism• Trt: surgical removal for
denture construction
Exostoses
Buccal exostosis
Subpontine exostosis
Palatal exostosis
Exostosis
• Histopathology
Dense Bone Island
• Sclerotic bone• Premolar region of
the mandible• Well defined not
surrounded by radiolucent line
• Separated or attached to root apex
Tumors of Bone
• Source: marrow, cartilage, bone, fibrous, vascular
• Benign and malignant
Benign tumors
• Osteoma
• Osteoblastoma
• Chondroma
Osteoma
• Benign slow growing tumor
• Mature bone
• Mandible>maxilla
• Sub periosteal or central
• D/D: dense bone island, exostosis
Histology: compact or cancellous
Osteoma
• Multiple osteomas of the jaws in Gardner syndrome– AD– Polyposis coli marked tendency for malignant
change– Fibrous tumors– Sebacous cysts of the skin– Multiple impacted and supernumerary teeth
Osteoblastoma
• Histologically and radiographically resembling cementoblastoma
– Not related to
the roots of teeth
Cementoblastoma
Osteosarcoma
• 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
• Increased incidence in Paget’s disease
Osteosarcoma• Radiographic Features
– Radiolucent, radiopaque, or mixed– Margins are poorly defined– “Sun-ray” appearance: bony trabeculae
radiating from periphery or periosteum. Present in only 25% of cases and not unique to osteosarcoma.
– Early feature: localized symmetrical widening of periodontal ligament space
Osteosarcoma
Osteosarcoma• Microscopic: malignant osteoblasts and
direct formation of abnormal osteoid and bone
• Treatment and Prognosis– Radical surgery, radiation therapy,
chemotherapy– Mandibular lesions have better prognosis