Paget`s Disease of Bone

and 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 Disease
Pathogenesis
3 overlapping phases
2. Predominantly osteolytic phase
Increased osteoclastic resorption
3. Active stage of mixed osteolysis and
osteogenesis
Abnormal osteoblastic deposition and increased
vascularity
4. Sclerotic phase
Dense bone and decreased vascularity
 Paget’s Disease of Bone
Clinical 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 Bone
Clinical Features
• Enlargement of skull and
facial bones.

• Narrowing of skull
foramina may cause
cranial nerve dysfunction
 Paget’s Disease
Oral 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 Disease
Oral 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 Bone
Radiographic 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 Disease
Treatment 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 Granuloma
Clinical Features
• Probably reactive and not neoplastic
– Reactive to heamodynamic changes: trauma
or heamorrhage
• Most common in young adults
• Female predominance
• Only 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 Granuloma
Radiographic 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 Granuloma
Microscopic Features
• Fibrous connective tissue, multinucleated
giant cells, hemorrhage and hemosiderin
 1

2
 Central Giant Cell Granuloma
Treatment 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

Nodular torus Spindle torus and flat torus Lobular torus

• Can slowly increase in size

• Peak prevalence is in adult- dynamic
 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 Subpontine
exostosi exostosis
s

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