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BONE PATHOLOGYBONE PATHOLOGY
INDIAN DENTALACADEMYINDIAN DENTALACADEMY
Leader in continuing Dental EducationLeader in continuing Dental Education
www.indiandentalacademy.com
Osteogenesis ImperfectaOsteogenesis Imperfecta 11
 A heterogeneous group of heritableA heterogeneous group of heritable
disorders characterized by impairment ofdisorders characterized by impairment of
collagen maturation.collagen maturation.
 Mutation of COL1A1 (chromosome 17) orMutation of COL1A1 (chromosome 17) or
COL1A2 (7).COL1A2 (7).
 Most common type of heritable boneMost common type of heritable bone
disease (1/8000).disease (1/8000).
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Osteogenesis ImperfectaOsteogenesis Imperfecta 22
 Main features are bone fragility, blueMain features are bone fragility, blue
sclera, altered teeth, hypoacusis (hearingsclera, altered teeth, hypoacusis (hearing
loss) long bone and spine deformity andloss) long bone and spine deformity and
joint hyperextensibility.joint hyperextensibility.
 Oral findings include dentinogenesisOral findings include dentinogenesis
imperfecta, maxillary hypoplasia withimperfecta, maxillary hypoplasia with
class III malocclusion and radiographicclass III malocclusion and radiographic
lesions similar to florid cemento-osseouslesions similar to florid cemento-osseous
dysplasia.dysplasia.
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Indian Dental academy
• www.indiandentalacademy.com
• Leader continuing dental education
• Offer both online and offline dental courses
Osteogenesis ImperfectaOsteogenesis Imperfecta 33
 Type I: AD; Most common and mildestType I: AD; Most common and mildest
form, 10% congenital fracture; 10% neverform, 10% congenital fracture; 10% never
fracture; blue sclera; hearing loss beforefracture; blue sclera; hearing loss before
30; hyperextensibility and tooth defects30; hyperextensibility and tooth defects
variable.variable.
 Type II: AR/AD; Most severe form; 90%Type II: AR/AD; Most severe form; 90%
stillborn or die before 4 weeks; bluestillborn or die before 4 weeks; blue
sclera; opalescent teeth may be present.sclera; opalescent teeth may be present.
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Osteogenesis ImperfectaOsteogenesis Imperfecta 44
 Type III: AD/AR: Moderate to severe boneType III: AD/AR: Moderate to severe bone
fragility; blue sclera fades over time;fragility; blue sclera fades over time;
hyperextensibility and hearing loss common;hyperextensibility and hearing loss common;
2/3 die in childhood from cardiopulmonary2/3 die in childhood from cardiopulmonary
failure 2failure 2º to kyphoscoliosis; some haveº to kyphoscoliosis; some have
opalescent dentin.opalescent dentin.
 Type IV: AD; Mild to moderate bone fragility-Type IV: AD; Mild to moderate bone fragility-
50% congenital fractures with frequency50% congenital fractures with frequency
decreasing with time; blue sclera fades overdecreasing with time; blue sclera fades over
time; opalescent dentin variable.time; opalescent dentin variable.
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OsteopetrosisOsteopetrosis 11
(Albers-Sch(Albers-Schönberg Disease)önberg Disease)
 A group of rare hereditary skeletalA group of rare hereditary skeletal
defects of increased bone densitydefects of increased bone density
resulting from defective remodeling dueresulting from defective remodeling due
to failure in osteoclast function.to failure in osteoclast function.
 Endochondral, endosteal and periostealEndochondral, endosteal and periosteal
growth without concomitant resorptiongrowth without concomitant resorption
results in thickening of bone.results in thickening of bone.
 Incidence estimated between 1 inIncidence estimated between 1 in
100,000-500,000.100,000-500,000.
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OsteopetrosisOsteopetrosis 22
(Albers-Sch(Albers-Schönberg Disease)önberg Disease)
 Infantile (malignant form): AR; marrowInfantile (malignant form): AR; marrow
failure, fracture and cranial nervefailure, fracture and cranial nerve
compression (blindness, deafness,compression (blindness, deafness,
facial paralysis.facial paralysis.
 Hepatosplenomegaly, infections andHepatosplenomegaly, infections and
osteomyelitis.osteomyelitis.
 Delayed tooth eruption; extractionDelayed tooth eruption; extraction
osteomyelitis .osteomyelitis .
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OsteopetrosisOsteopetrosis 33
(Albers-Sch(Albers-Schönberg Disease)önberg Disease)
 Adult (benign form): AD; limited mostly to axialAdult (benign form): AD; limited mostly to axial
skeleton; bone pain is common but nearly 40%skeleton; bone pain is common but nearly 40%
are asymptomatic; marrow failure rare.are asymptomatic; marrow failure rare.
 Variants with cranial nerve compression-withoutVariants with cranial nerve compression-without
fracture or fracture without cranial nervefracture or fracture without cranial nerve
compression .compression .
 Treatment of infantile with BMT for cure;Treatment of infantile with BMT for cure;
interferon, calcitriol, steroids, PTH, MCSF,interferon, calcitriol, steroids, PTH, MCSF,
erythropoietin.erythropoietin.
 Supportive measures; transfusion, antibiotics,Supportive measures; transfusion, antibiotics,
surgery & hyperbaric oxygen.surgery & hyperbaric oxygen.www.indiandentalacademy.com
Cleidocranial DysplasiaCleidocranial Dysplasia 11
 Defect in CBFA1 gene (6p21); AD with 40%Defect in CBFA1 gene (6p21); AD with 40%
sporadic cases; defect in osteoblasticsporadic cases; defect in osteoblastic
differentiation and bone formation.differentiation and bone formation.
 Clavicles show hypoplasia or malformation;Clavicles show hypoplasia or malformation;
about 10% one/both clavicles are absent.about 10% one/both clavicles are absent.
 Short stature, large head with frontal & parietalShort stature, large head with frontal & parietal
bossing, hypertelorism, broad nose, depressedbossing, hypertelorism, broad nose, depressed
nasal bridge.nasal bridge.
 Persistent open fontanels and cranial sutures.Persistent open fontanels and cranial sutures.
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Cleidocranial DysplasiaCleidocranial Dysplasia 22
 Dental & gnathic manifestations includeDental & gnathic manifestations include
narrow, high-arched palate w/o cleft,narrow, high-arched palate w/o cleft,
prolonged retention of deciduous teeth,prolonged retention of deciduous teeth,
delayed eruption of permanent teeth,delayed eruption of permanent teeth,
numerous unerupted permanent andnumerous unerupted permanent and
supernumerary teeth (lack 2supernumerary teeth (lack 2º cementum)º cementum)..
Narrow ramus, pointed coronoid processes,Narrow ramus, pointed coronoid processes,
thin zygomatic arch, small/absent maxillarythin zygomatic arch, small/absent maxillary
sinuses, acute gonial angle, prognathismsinuses, acute gonial angle, prognathism..
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Focal OsteoporoticFocal Osteoporotic
Marrow DefectMarrow Defect
 Non-pathologic area of hematopoietic marrowNon-pathologic area of hematopoietic marrow
sufficient in size to appear radiographically assufficient in size to appear radiographically as
a radiolucency.a radiolucency.
 Theories as to cause include aberrant boneTheories as to cause include aberrant bone
regeneration following tooth extraction,regeneration following tooth extraction,
persistent fetal marrow or marrow hypoplasiapersistent fetal marrow or marrow hypoplasia
due to increased demand for erythrocytes.due to increased demand for erythrocytes.
 Asymptomatic ill-defined circumscribedAsymptomatic ill-defined circumscribed
lucency from a few to several cm in size.lucency from a few to several cm in size.
 More than 75% of cases in adult females in anMore than 75% of cases in adult females in an
edentulous area posterior mandible; biopsy.edentulous area posterior mandible; biopsy.www.indiandentalacademy.com
IdiopathicIdiopathic
OsteosclerosisOsteosclerosis 11
 Focal area of increased radiodensity ofFocal area of increased radiodensity of
unknown cause.unknown cause.
 Also known as dense bone island, boneAlso known as dense bone island, bone
eburnation, bone whorl, bone scar, enostosis,eburnation, bone whorl, bone scar, enostosis,
focal (periapical) osteopetrosis.focal (periapical) osteopetrosis.
 Peak age 20’s; no sex predilection.Peak age 20’s; no sex predilection.
 Well defined rounded or elliptic radiodenseWell defined rounded or elliptic radiodense
mass 3-20 mm.mass 3-20 mm.
 Common (5%) in mandible (90%); first molar, 2Common (5%) in mandible (90%); first molar, 2ndnd
premolar or 2premolar or 2ndnd
molar; most are periapical.molar; most are periapical.
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IdiopathicIdiopathic
OsteosclerosisOsteosclerosis 22
 Need to rule-out condensing osteitis (pulpNeed to rule-out condensing osteitis (pulp
disease).disease).
 Diagnosis based on history clinical andDiagnosis based on history clinical and
radiographic features; pre-existingradiographic features; pre-existing
radiographs and periodic reassessment.radiographs and periodic reassessment.
 Biopsy if expansion is present.Biopsy if expansion is present.
 Because lesion becomes stable and indolent,Because lesion becomes stable and indolent,
not treatment necessary.not treatment necessary.
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Massive OsteolysisMassive Osteolysis 11
 Also known as Gorham disease, Gorham-Also known as Gorham disease, Gorham-
Stout syndrome, vanishing bone disease andStout syndrome, vanishing bone disease and
phantom bone disease.phantom bone disease.
 Rare, idiopathic spontaneous progressiveRare, idiopathic spontaneous progressive
destruction of one or more bones; maydestruction of one or more bones; may
represent hamangiomatosis of bone.represent hamangiomatosis of bone.
 Most often in children & young adults; 50%Most often in children & young adults; 50%
report antecedent trauma.report antecedent trauma.
 Pelvis, humeral head or shaft & axial skeletonPelvis, humeral head or shaft & axial skeleton
most common sites.most common sites.
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Massive OsteolysisMassive Osteolysis 22
 30% maxillofacial involvement, usually30% maxillofacial involvement, usually
the mandible.the mandible.
 Signs include mobile teeth, pain,Signs include mobile teeth, pain,
malocclusion, midline deviation,malocclusion, midline deviation,
pathologic fracture and obstructive sleeppathologic fracture and obstructive sleep
apnea.apnea.
 Intramedullary lucency, loss of laminaIntramedullary lucency, loss of lamina
dura, thinning of cortex.dura, thinning of cortex.
 Spontaneous arrest; radiation/surgery.Spontaneous arrest; radiation/surgery.www.indiandentalacademy.com
Paget’s Disease of BonePaget’s Disease of Bone 11
 Also known as osteitis deformansAlso known as osteitis deformans
 Idiopathic disorder of abnormal anarchicIdiopathic disorder of abnormal anarchic
resorption and deposition of boneresorption and deposition of bone
(abnormal remodeling).(abnormal remodeling).
 Relatively common after age 40; 1/100-Relatively common after age 40; 1/100-
150 (increasing to 1/10 by age 90); M>F;150 (increasing to 1/10 by age 90); M>F;
white > blacks or Asians.white > blacks or Asians.
 Usually polyostotic (multiple boneUsually polyostotic (multiple bone
affected), rarely monostotic (single bone)affected), rarely monostotic (single bone)www.indiandentalacademy.com
Paget’s Disease of BonePaget’s Disease of Bone 22
 Lumbar vertebrae, pelvis, skull & femur; jawLumbar vertebrae, pelvis, skull & femur; jaw
involvement in 17%, usually the maxilla.involvement in 17%, usually the maxilla.
 Bowing of legs, leontiasis ossea, fracture,Bowing of legs, leontiasis ossea, fracture,
bone and joint pain, pressure neuropathy.bone and joint pain, pressure neuropathy.
 May cause progressive alveolar ridgeMay cause progressive alveolar ridge
expansion, spacing of teeth and “dentalexpansion, spacing of teeth and “dental
appliances that no longer fit the mouth”.appliances that no longer fit the mouth”.
 Altered trabecular pattern initially fromAltered trabecular pattern initially from
decreased bone density evolves to patchydecreased bone density evolves to patchy
sclerotic areas having a “cotton wool”sclerotic areas having a “cotton wool”
appearance.appearance. www.indiandentalacademy.com
Paget’s Disease of BonePaget’s Disease of Bone 33
 Alternating resorption and formation of boneAlternating resorption and formation of bone
produces a characteristic “mosaic”pattern andproduces a characteristic “mosaic”pattern and
marrow replaced by highly vascular fibrousmarrow replaced by highly vascular fibrous
connective tissue.connective tissue.
 Elevation of serum alkaline phosphataseElevation of serum alkaline phosphatase
treated if elevated by more than 25-50%;treated if elevated by more than 25-50%;
elevated urinary hydroxyproline levels.elevated urinary hydroxyproline levels.
 Increased risk of osteosarcoma (1%) and giantIncreased risk of osteosarcoma (1%) and giant
cell tumors of bone.cell tumors of bone.
 Treatment with calcitonin & biophosphonate;Treatment with calcitonin & biophosphonate;
plicamycin for refractory casesplicamycin for refractory caseswww.indiandentalacademy.com
Central Giant CellCentral Giant Cell
Granuloma (Lesion)Granuloma (Lesion) 11
 A non-neoplastic lesion of the jaws; mostA non-neoplastic lesion of the jaws; most
common before age 30 (60%); females>males;common before age 30 (60%); females>males;
70% in mandible, especially anterior two-thirds70% in mandible, especially anterior two-thirds
 Most often asymptomatic or produce painlessMost often asymptomatic or produce painless
expansion; occasionally pain, paresthesia orexpansion; occasionally pain, paresthesia or
cortical perforation.cortical perforation.
 Non-aggressive lesions are most common,Non-aggressive lesions are most common,
produce few symptoms and are slow growing.produce few symptoms and are slow growing.
 Aggressive lesions produce pain, rapidAggressive lesions produce pain, rapid
growth, cortical perforation and rootgrowth, cortical perforation and root
resorption.resorption. www.indiandentalacademy.com
Central Giant CellCentral Giant Cell
Granuloma (Lesion)Granuloma (Lesion) 22
 Radiographic lesions range from less than 5Radiographic lesions range from less than 5
mm to greater than 10 cm; unilocular ormm to greater than 10 cm; unilocular or
multilocular; differential diagnosis required.multilocular; differential diagnosis required.
 Histopathologically consist ofHistopathologically consist of
multinucleated giant cells in a stroma ofmultinucleated giant cells in a stroma of
spindle-shaped mesenchymal cells usuallyspindle-shaped mesenchymal cells usually
with numerous small vessel and hemorrhagewith numerous small vessel and hemorrhage
—indistinguishable from—indistinguishable from
hyperparathyroidism, cherubism andhyperparathyroidism, cherubism and
aneurysmal bone cyst.aneurysmal bone cyst.
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Central Giant CellCentral Giant Cell
Granuloma (Lesion)Granuloma (Lesion) 33
 R/O other causes for “giant cell lesions”R/O other causes for “giant cell lesions”
 Treatment by thorough curettage;Treatment by thorough curettage;
recurrence rate 15-20%; resection forrecurrence rate 15-20%; resection for
larger lesions.larger lesions.
 Investigational treatments of repeatedInvestigational treatments of repeated
intrabony lesional injection ofintrabony lesional injection of
triamcinolone or daily calcitonintriamcinolone or daily calcitonin
injection (or nasal spray) for one year asinjection (or nasal spray) for one year as
alternatives to deforming surgery.alternatives to deforming surgery.
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Giant Cell TumorGiant Cell Tumor
 Considered a true neoplasm usuallyConsidered a true neoplasm usually
found in long bones.found in long bones.
 Although it can be histopathologicallyAlthough it can be histopathologically
indistinguishable from central giant cellindistinguishable from central giant cell
granuloma, the biologic behavior of GCTgranuloma, the biologic behavior of GCT
different from that of CGCG; has adifferent from that of CGCG; has a
higher recurrence rate and 10% rate ofhigher recurrence rate and 10% rate of
metastasize.metastasize.
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CherubismCherubism 11
 Rare development condition of the jaws, generallyRare development condition of the jaws, generally
AD (4p16) with high penetrance but variableAD (4p16) with high penetrance but variable
expressivity.expressivity.
 Onset usually 2-5; progresses until puberty,Onset usually 2-5; progresses until puberty,
becomes stable, then slowly regresses.becomes stable, then slowly regresses.
 Mandibular involvement of posterior areas resultsMandibular involvement of posterior areas results
in bilateral symmetrical multilocular expansion;in bilateral symmetrical multilocular expansion;
maxillary involvement usually in tuberosity areas.maxillary involvement usually in tuberosity areas.
 Involved bone produces widening and distortion ofInvolved bone produces widening and distortion of
alveolar ridges, tooth displacement, alteredalveolar ridges, tooth displacement, altered
eruption, impaired mastication & speecheruption, impaired mastication & speech
difficulties.difficulties. www.indiandentalacademy.com
CherubismCherubism 22
 Clavicle, ribs and humerus reported as rareClavicle, ribs and humerus reported as rare
extragnathic sites.extragnathic sites.
 Microscopically similar to CGCG;Microscopically similar to CGCG;
eosinophilic cuff-like deposits around smalleosinophilic cuff-like deposits around small
vessels a helpful finding. Long-standingvessels a helpful finding. Long-standing
lesions become more fibrous with fewerlesions become more fibrous with fewer
giant cells.giant cells.
 Prognosis variable; return to normalcy forPrognosis variable; return to normalcy for
many; some with significant deformity;many; some with significant deformity;
dental management sometimes difficult.dental management sometimes difficult.
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Simple Bone CystSimple Bone Cyst 11
 Also known as traumatic bone cyst,Also known as traumatic bone cyst,
hemorrhagic bone cyst, solitary bonehemorrhagic bone cyst, solitary bone
cyst & idiopathic bone cavity.cyst & idiopathic bone cavity.
 A benign, empty or fluid-filled cavityA benign, empty or fluid-filled cavity
within bone devoid of epithelial lining.within bone devoid of epithelial lining.
 Trauma might play role in some cases butTrauma might play role in some cases but
other theories are offered.other theories are offered.
 Majority occur in long bones, ages 10-20;Majority occur in long bones, ages 10-20;
mandible; 60% of jaw lesions in males.mandible; 60% of jaw lesions in males.www.indiandentalacademy.com
Simple Bone CystSimple Bone Cyst 22
 Usually asymptomatic; 20% have swelling ofUsually asymptomatic; 20% have swelling of
jaw.jaw.
 Well-delineated radiolucency; usually inWell-delineated radiolucency; usually in
premolar-molar region, often scalloped upwardpremolar-molar region, often scalloped upward
between the roots of teeth; rarely multilocular.between the roots of teeth; rarely multilocular.
 Pulp test teeth; can be associated withPulp test teeth; can be associated with
cemento-osseous dysplasia.cemento-osseous dysplasia.
 Diagnosis based on clinical, radiographic andDiagnosis based on clinical, radiographic and
surgical findings.surgical findings.
 Gnathic lesions treated by surgical explorationGnathic lesions treated by surgical exploration
and follow-up.and follow-up.www.indiandentalacademy.com
Aneurysmal Bone CystAneurysmal Bone Cyst 11
 A non-neoplastic lesion of uncertain etiologyA non-neoplastic lesion of uncertain etiology
that may mimic the growth and behavior of athat may mimic the growth and behavior of a
neoplasm of bone. It is not a “true cyst.”neoplasm of bone. It is not a “true cyst.”
 Trauma, vascular malformation or anTrauma, vascular malformation or an
associated neoplasm that disrupts the normalassociated neoplasm that disrupts the normal
hemodynamics of bone have been proposedhemodynamics of bone have been proposed
as causes.as causes.
 An intraosseous accumulation of variable-An intraosseous accumulation of variable-
sized blood-filled spaces surrounded bysized blood-filled spaces surrounded by
cellular fibrous CT & reactive bone.cellular fibrous CT & reactive bone.
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Aneurysmal Bone CystAneurysmal Bone Cyst 22
 Most common in shaft of long bone; ageMost common in shaft of long bone; age
<30; gnathic lesions account for 2% with a<30; gnathic lesions account for 2% with a
mean age of 20 years; most often inmean age of 20 years; most often in
posterior mandible.posterior mandible.
 Rapid swelling, pain often reported.Rapid swelling, pain often reported.
 Unilocular or multilocular radiolucencyUnilocular or multilocular radiolucency
with cortical expansion; ballooning “blow-with cortical expansion; ballooning “blow-
out” distension of bone contour;out” distension of bone contour;
sometimes radiopaque foci or smallsometimes radiopaque foci or small
trabaculae seen.trabaculae seen.www.indiandentalacademy.com
Aneurysmal Bone CystAneurysmal Bone Cyst 33
 At surgery lesion often describe as aAt surgery lesion often describe as a
“blood-soaked sponge”.“blood-soaked sponge”.
 20% of jaw lesions associated with other20% of jaw lesions associated with other
pathosis (fibro-osseous lesion of CGCG).pathosis (fibro-osseous lesion of CGCG).
 Curettage or enucleation; recurrenceCurettage or enucleation; recurrence
variable (8-60%); resection rarelyvariable (8-60%); resection rarely
needed.needed.
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Fibro-osseous LesionsFibro-osseous Lesions
of the Jawsof the Jaws
Fibrous dysplasiaFibrous dysplasia
Cemento-osseous dysplasiaCemento-osseous dysplasia
a.a. Focal cemento-osseous dysplasiaFocal cemento-osseous dysplasia
b.b. Periapical cemento-osseous dysplasiaPeriapical cemento-osseous dysplasia
c.c. Florid cemento-osseous dysplasiaFlorid cemento-osseous dysplasia
•Ossifying fibromaOssifying fibroma
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Fibrous DysplasiaFibrous Dysplasia 11
 Developmental condition of replacement ofDevelopmental condition of replacement of
normal bone by an excessive proliferation ofnormal bone by an excessive proliferation of
cellular fibrous connective tissue intermixedcellular fibrous connective tissue intermixed
with irregular bone trabeculae.with irregular bone trabeculae.
 Sporadic condition attributed to mutation ofSporadic condition attributed to mutation of
GNAS 1 gene (guanine nucleotide-binding,GNAS 1 gene (guanine nucleotide-binding, --
stimulating activity peptide); clinical severitystimulating activity peptide); clinical severity
determined by time when the mutation occurs;determined by time when the mutation occurs;
early embryonic mutation produces polyostoticearly embryonic mutation produces polyostotic
disease with other abnormalities; postnataldisease with other abnormalities; postnatal
monostotic disease.monostotic disease.
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Fibrous DysplasiaFibrous Dysplasia 22
 Monostotic accounts for 80-85% of cases; jawsMonostotic accounts for 80-85% of cases; jaws
are common location; maxilla > mandible;are common location; maxilla > mandible;
maxillary lesions sometimes part of widermaxillary lesions sometimes part of wider
craniofacial fibrous dysplasia.craniofacial fibrous dysplasia.
 Radiographic poorly demarcated “ground-Radiographic poorly demarcated “ground-
glass” appearance of affected bone causingglass” appearance of affected bone causing
expansion; in long bones often radiolucent,expansion; in long bones often radiolucent,
multilocular & expansile.multilocular & expansile.
 PDL may be narrow and lamina dura indistinct;PDL may be narrow and lamina dura indistinct;
inferior alveolar nerve canal displacedinferior alveolar nerve canal displaced
superiorly.superiorly. www.indiandentalacademy.com
Fibrous DysplasiaFibrous Dysplasia 33
 Polyostotic (PFD) involves two or morePolyostotic (PFD) involves two or more
bones.bones.
 Jaffe-Lichtenstein syndrome consists ofJaffe-Lichtenstein syndrome consists of
PFD withPFD with café au laitcafé au lait pigmentation.pigmentation.
 McCune-Albright syndrome consists ofMcCune-Albright syndrome consists of
PFD,PFD, café au laitcafé au lait pigmentation andpigmentation and
multiple endocrinopathies such a sexualmultiple endocrinopathies such a sexual
precocity, pituitary adenoma orprecocity, pituitary adenoma or
hyperthyroidism.hyperthyroidism.
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Fibrous DysplasiaFibrous Dysplasia 44
 Histopathologically consists ofHistopathologically consists of
trabeculae of woven bone in a cellular,trabeculae of woven bone in a cellular,
fibrous stroma (Chinese characters).fibrous stroma (Chinese characters).
 Excision or resection of small jawExcision or resection of small jaw
lesions; extensive or diffuse lesions maylesions; extensive or diffuse lesions may
require repeated recontouring forrequire repeated recontouring for
cosmesis; 25-50% regrowth after shave-cosmesis; 25-50% regrowth after shave-
down.down.
 Occasional development ofOccasional development of
osteosarcoma in bone affected by FD.osteosarcoma in bone affected by FD.www.indiandentalacademy.com
Cemento-OsseousCemento-Osseous
DysplasiasDysplasias 11
 Non-neoplastic fibro-osseous alteration ofNon-neoplastic fibro-osseous alteration of
bone in the tooth bearing areas of the jaws.bone in the tooth bearing areas of the jaws.
 Three distinct radiographic/clinical patternsThree distinct radiographic/clinical patterns
that share a common histopathologicthat share a common histopathologic
appearance.appearance.
 Cellular connective tissue containing aCellular connective tissue containing a
mixture of woven bone, lamellar bone andmixture of woven bone, lamellar bone and
cementum-like particles; ratio of fibrouscementum-like particles; ratio of fibrous
tissue decreases over time as it becomestissue decreases over time as it becomes
sclerotic by fusion of calcified elements.sclerotic by fusion of calcified elements.
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Cemento-OsseousCemento-Osseous
DysplasiasDysplasias (Focal)(Focal)22
 Focal cemento-osseous dysplasiaFocal cemento-osseous dysplasia occursoccurs
most frequently in young to middle aged whitemost frequently in young to middle aged white
females (9:1,F:M).females (9:1,F:M).
 Solitary radiolucent to radiopaque lesion, lessSolitary radiolucent to radiopaque lesion, less
than 1.5 cm, if opaque with thin radiolucentthan 1.5 cm, if opaque with thin radiolucent
rim; most often in posterior mandible; well-rim; most often in posterior mandible; well-
defined with slight irregular borders.defined with slight irregular borders.
 Easily fragmented gritty tissue that can beEasily fragmented gritty tissue that can be
curetted but does not separate easily from thecuretted but does not separate easily from the
surrounding bone.surrounding bone.
 Monitor once diagnosis established by biopsy.Monitor once diagnosis established by biopsy.www.indiandentalacademy.com
Cemento-OsseousCemento-Osseous
Dysplasias (Periapical)Dysplasias (Periapical) 33
 Periapical cemento-osseous dysplasiaPeriapical cemento-osseous dysplasia occursoccurs
most frequently in black females (70%) 30-50most frequently in black females (70%) 30-50
y.o.y.o.
 F:M, 14:1.F:M, 14:1.
 Asymptomatic, anterior mandible, typicallyAsymptomatic, anterior mandible, typically
multifocal, periapical to vital teeth. Must pulpmultifocal, periapical to vital teeth. Must pulp
test teeth.test teeth.
 Lucent > opaque over time; < 1cm; PDL intact,Lucent > opaque over time; < 1cm; PDL intact,
opacity not fused to tooth.opacity not fused to tooth.
 No treatment necessary.No treatment necessary.
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Cemento-OsseousCemento-Osseous
Dysplasias (Florid)Dysplasias (Florid) 33
 Florid cemento-osseous dysplasiaFlorid cemento-osseous dysplasia occurs mostoccurs most
frequently in middle aged to older black females (90%).frequently in middle aged to older black females (90%).
 Multifocal involvement but not limited to the anteriorMultifocal involvement but not limited to the anterior
mandible; often bilaterally symmetric.mandible; often bilaterally symmetric.
 Most often asymptomatic; pain, swelling or signs ofMost often asymptomatic; pain, swelling or signs of
osteomyelitis.osteomyelitis.
 Lucent/opaque; sometimes associated with simpleLucent/opaque; sometimes associated with simple
bone cyst.bone cyst.
 No treatment for asymptomatic lesions; exploration ofNo treatment for asymptomatic lesions; exploration of
simple bone cysts; avoid surgical exposure of affectedsimple bone cysts; avoid surgical exposure of affected
sclerotic areas; symptomatic areas are treated thesclerotic areas; symptomatic areas are treated the
same as for osteomyelitis.same as for osteomyelitis.
www.indiandentalacademy.com
Familial GigantiformFamilial Gigantiform
CementomaCementoma
 Uncommon hereditary AD disorder of gnathicUncommon hereditary AD disorder of gnathic
bone in which massive sclerotic masses ofbone in which massive sclerotic masses of
disorganized mineralized tissue develop.disorganized mineralized tissue develop.
 Begin as multiple periapical lucencies whichBegin as multiple periapical lucencies which
expand to produce a mixed radiolucent-expand to produce a mixed radiolucent-
radiopaque pattern.radiopaque pattern.
 Anemia and multiple polypoid adenomas of theAnemia and multiple polypoid adenomas of the
uterus reported in some affected females.uterus reported in some affected females.
 Extensive resection & reconstruction may beExtensive resection & reconstruction may be
needed to achieve acceptable cosmesis.needed to achieve acceptable cosmesis.
www.indiandentalacademy.com
Ossifying FibromaOssifying Fibroma
 AA true neoplasm of an admixture of fibrous tissue &true neoplasm of an admixture of fibrous tissue &
bony trabeculae, cementum-like spherules, or both.bony trabeculae, cementum-like spherules, or both.
 Most frequent in third-fourth decade; femaleMost frequent in third-fourth decade; female
predilection.predilection.
 Premolar-molar region of mandible most frequent site.Premolar-molar region of mandible most frequent site.
 Small lesions asymptomatic; larger lesions produceSmall lesions asymptomatic; larger lesions produce
painless swelling.painless swelling.
 Well defined radiolucency, often with a scleroticWell defined radiolucency, often with a sclerotic
border, containing varying amounts of opacity. Largerborder, containing varying amounts of opacity. Larger
lesions produce downward bow of the inferior cortexlesions produce downward bow of the inferior cortex
of mandible.of mandible.
 Enucleation; resection for large lesions.Enucleation; resection for large lesions.www.indiandentalacademy.com
Juvenile OssifyingJuvenile Ossifying
FibromaFibroma 11
 Differs from common OF by ageDiffers from common OF by age
(younger), location (maxilla) & biologic(younger), location (maxilla) & biologic
behavior (sometime aggressive and/orbehavior (sometime aggressive and/or
recurrent).recurrent).
 Two microscopic types; trabecular &Two microscopic types; trabecular &
psammomatoid; age at diagnosis 11 andpsammomatoid; age at diagnosis 11 and
22 respectively.22 respectively.
 Maxilla often with impingement onMaxilla often with impingement on
sinuses, orbit, nose and cranium.sinuses, orbit, nose and cranium.
www.indiandentalacademy.com
Juvenile OssifyingJuvenile Ossifying
FibromaFibroma 22
 Richly cellular fibrous tissue showingRichly cellular fibrous tissue showing
strand of osteoid or psammomatoidstrand of osteoid or psammomatoid
ossicles.ossicles.
 Growth of individual tumors varies fromGrowth of individual tumors varies from
slow to rapid; tends to be rapid inslow to rapid; tends to be rapid in
younger patients.younger patients.
 Complete curettage for small lesions;Complete curettage for small lesions;
resection for larger, aggressive lesions;resection for larger, aggressive lesions;
recurrence 30-58%.recurrence 30-58%.
www.indiandentalacademy.com
OsteomaOsteoma
 Benign tumors (neoplasm?) of mature compact orBenign tumors (neoplasm?) of mature compact or
cancellous bone that are restricted to the craniofacialcancellous bone that are restricted to the craniofacial
skeleton.skeleton.
 May be periosteal or endoseal; perioseal lesionsMay be periosteal or endoseal; perioseal lesions
produce slowing growing mass; endosteal lesionsproduce slowing growing mass; endosteal lesions
asymptomatic or slow enlargement.asymptomatic or slow enlargement.
 Condylar lesion produces lobulated mass; deviation ofCondylar lesion produces lobulated mass; deviation of
midline.midline.
 Radiographically a circumscribed sclerotic massRadiographically a circumscribed sclerotic mass
 Symptomatic or deforming lesions removed bySymptomatic or deforming lesions removed by
conservative excision.conservative excision.
www.indiandentalacademy.com
Gardner SyndromeGardner Syndrome
 Rare inherited AD disorder (gene on chromosome 5)Rare inherited AD disorder (gene on chromosome 5)
 Within the spectrum of familiar colorectal polyposisWithin the spectrum of familiar colorectal polyposis
 1 in 8,300-16,0001 in 8,300-16,000
 Osteomas of any bone, skull, paranasal sinuses andOsteomas of any bone, skull, paranasal sinuses and
mandible common.mandible common.
 Supernumerary teeth in 20%Supernumerary teeth in 20%
 Epidermoid cysts of skin, desmoid tumor of skin,Epidermoid cysts of skin, desmoid tumor of skin,
adenomatous colonic polyps > colon cancer,adenomatous colonic polyps > colon cancer,
pigmented lesions of ocular fundus, 100-fold increasepigmented lesions of ocular fundus, 100-fold increase
in thyroid cancer in females.in thyroid cancer in females.
 Colonectomy; elective prophylactic thyroidectomy,Colonectomy; elective prophylactic thyroidectomy,
elective removal of osteomas; cysts and other tumors.elective removal of osteomas; cysts and other tumors.www.indiandentalacademy.com
Osteoblastoma &Osteoblastoma &
Osteoid OsteomaOsteoid Osteoma
 Rare neoplasms of bone (<1%) are microscopicallyRare neoplasms of bone (<1%) are microscopically
identical; osteoblastomas most frequent in vertebralidentical; osteoblastomas most frequent in vertebral
column, sacrum, calvarium, long bones small bones ofcolumn, sacrum, calvarium, long bones small bones of
hands/feet; osteoid osteoma most often femur, tibia &hands/feet; osteoid osteoma most often femur, tibia &
phalanges.phalanges.
 Osteoid osteoma produce prostaglandin > pain relievedOsteoid osteoma produce prostaglandin > pain relieved
by aspirin; not osteoblastoma.by aspirin; not osteoblastoma.
 In jaws, slight mandible predilection in posterior area;In jaws, slight mandible predilection in posterior area;
85% < age 30.85% < age 30.
 Painful radiolucent/opaque lesion often 2-4 cm.Painful radiolucent/opaque lesion often 2-4 cm.
 Local excision or curettage; 50% chance for recurrenceLocal excision or curettage; 50% chance for recurrence
of aggressive lesions.of aggressive lesions.www.indiandentalacademy.com
CementoblastomaCementoblastoma
 Rare neoplasm thought by some to be identical withRare neoplasm thought by some to be identical with
osteoblastoma differing only by its attachment to theosteoblastoma differing only by its attachment to the
root of a tooth. <1% of odontogenic tumor.root of a tooth. <1% of odontogenic tumor.
 75% in mandible; 90% molar or premolar with 50%75% in mandible; 90% molar or premolar with 50%
involving first molar.involving first molar.
 Children and young adults; 50% <age 20 & 75% < ageChildren and young adults; 50% <age 20 & 75% < age
30.30.
 Pain reported by 2/3Pain reported by 2/3
 Opaque mass fused to root surrounded by thinOpaque mass fused to root surrounded by thin
radiolucent rim.radiolucent rim.
 Extraction/removal of mass; removal of mass, rootExtraction/removal of mass; removal of mass, root
amputation & endodontics.amputation & endodontics.www.indiandentalacademy.com
ChondromaChondroma
 Benign neoplasm of hyaline cartilage.Benign neoplasm of hyaline cartilage.
 Most common in the short tubular bonesMost common in the short tubular bones
of the hands and feet; multiple lesion inof the hands and feet; multiple lesion in
Ollier disease or Maffucci syndrome; jawOllier disease or Maffucci syndrome; jaw
lesions are uncommon and most are inlesions are uncommon and most are in
condyle or anterior maxilla.condyle or anterior maxilla.
 Radiolucent with central area of opacityRadiolucent with central area of opacity
 Surgical removal; microscopic distinctionSurgical removal; microscopic distinction
from low-grade chondrosarcoma difficult.from low-grade chondrosarcoma difficult.www.indiandentalacademy.com
Chondromyxoid FibromaChondromyxoid Fibroma
 Uncommon benign neoplasm (<1%) most oftenUncommon benign neoplasm (<1%) most often
involving metaphysis of long bones; jawinvolving metaphysis of long bones; jaw
lesions are rare.lesions are rare.
 For jaw lesions average age 30; 75% inFor jaw lesions average age 30; 75% in
mandible; pain and swelling most commonmandible; pain and swelling most common
symptoms.symptoms.
 Circumscribed radiolucency with sclerotic orCircumscribed radiolucency with sclerotic or
scalloped margin; size range 1-6.5 cmscalloped margin; size range 1-6.5 cm
 Can be confused with chondrosarcomaCan be confused with chondrosarcoma
histologically.histologically.
 Curettage (25% recur) or surgical resection.Curettage (25% recur) or surgical resection.www.indiandentalacademy.com
SynovialSynovial
ChondromatosisChondromatosis
 Rare benign, non-neoplastic arthropathy ofRare benign, non-neoplastic arthropathy of
unknown cause; cartilaginous nodules formunknown cause; cartilaginous nodules form
within the synovial membrane.within the synovial membrane.
 Most often affects a single large joint; knee,Most often affects a single large joint; knee,
elbow, hip and shoulder most common;elbow, hip and shoulder most common;
mandible is rare site.mandible is rare site.
 Radiographically “loose bodies” in jointRadiographically “loose bodies” in joint
appear as round irregular radiopacities;appear as round irregular radiopacities;
widened joint space, irregular condylar headwidened joint space, irregular condylar head
 Removal of loose bodies; total synovectomy;Removal of loose bodies; total synovectomy;
menisectomy.menisectomy.www.indiandentalacademy.com
Desmoplastic FibromaDesmoplastic Fibroma
 Uncommon bone tumor of fibroblastic tissueUncommon bone tumor of fibroblastic tissue
 Metaphyseal region of humerus or tibia mostMetaphyseal region of humerus or tibia most
common; for jaw lesions, 90% in molar-ramuscommon; for jaw lesions, 90% in molar-ramus
region of mandible.region of mandible.
 Most case before age 30; for jaws the mean age isMost case before age 30; for jaws the mean age is
14.14.
 Painless swelling with radiolucency of bone.Painless swelling with radiolucency of bone.
 Composed of bland collagenous connectiveComposed of bland collagenous connective
tissue.tissue.
 Sometimes locally destructive and aggressive;Sometimes locally destructive and aggressive;
managed surgically according to aggressiveness.managed surgically according to aggressiveness.www.indiandentalacademy.com
OsteosarcomaOsteosarcoma 11
 Most common primary malignancy ofMost common primary malignancy of
bone (non-hematopoietic).bone (non-hematopoietic).
 Most are intramedullary; small number areMost are intramedullary; small number are
peripheral (juxtacortical), periosteal orperipheral (juxtacortical), periosteal or
parosteal.parosteal.
 Most often between age 10-20 and locatedMost often between age 10-20 and located
in distal femur or proximal tibia;in distal femur or proximal tibia;
secondary group > age 50 associated withsecondary group > age 50 associated with
Paget’s disease located in axial skeleton.Paget’s disease located in axial skeleton.
www.indiandentalacademy.com
OsteosarcomaOsteosarcoma 22
 Osteosarcoma of jaws accounts for 6-8%Osteosarcoma of jaws accounts for 6-8%
of all osteosarcomas; mean age of 33,of all osteosarcomas; mean age of 33,
maxilla = mandible.maxilla = mandible.
 Swelling and pain, loosening of teeth,Swelling and pain, loosening of teeth,
paresthesia and nasal obstruction mostparesthesia and nasal obstruction most
common in jaw lesions.common in jaw lesions.
 Osteoblastic, chondroblastic andOsteoblastic, chondroblastic and
fibroblastic are the most commonfibroblastic are the most common
microscopic subtypes.microscopic subtypes.
www.indiandentalacademy.com
OsteosarcomaOsteosarcoma 33
 Radiographic lesions of jaws vary fromRadiographic lesions of jaws vary from
lucent to opaque, often ill-defined;lucent to opaque, often ill-defined;
spiking or roots, widening of PDLspiking or roots, widening of PDL
(common); osteophytic reaction (sun-(common); osteophytic reaction (sun-
ray) is seen in 25%.ray) is seen in 25%.
 Prognosis linked to the initial attempt atPrognosis linked to the initial attempt at
complete removal.complete removal.
 Metastasis to regional nodes, lung andMetastasis to regional nodes, lung and
brain; > for long bones than jaws.brain; > for long bones than jaws.
www.indiandentalacademy.com
OsteosarcomaOsteosarcoma 44
 For extragnathic sites, pre-op (neoadjuvant)For extragnathic sites, pre-op (neoadjuvant)
chemotherapy followed by radical excisionchemotherapy followed by radical excision
and post-op (adjuvant) chemotherapy hasand post-op (adjuvant) chemotherapy has
increased survival to more than 4 years forincreased survival to more than 4 years for
80%.80%.
 Data for jaws sparse estimated 30-50%Data for jaws sparse estimated 30-50%
survival. Better outcomes for juxtacorticalsurvival. Better outcomes for juxtacortical
tumors compared to intramedullary lesions.tumors compared to intramedullary lesions.
 0.2% risk of post-radiation sarcoma for dose0.2% risk of post-radiation sarcoma for dose
or 7000 cGy.or 7000 cGy. www.indiandentalacademy.com
ChondrosarcomaChondrosarcoma 11
 Malignant neoplasm of bone (10%)Malignant neoplasm of bone (10%)
characterized by the formation of cartilage.characterized by the formation of cartilage.
 Jaw lesions are rare accounting for 1-3% ofJaw lesions are rare accounting for 1-3% of
all chondrosarcomas.all chondrosarcomas.
 Extragnathic lesions occur predominatelyExtragnathic lesions occur predominately
in those > age 50; ileum, femur & humerusin those > age 50; ileum, femur & humerus
most common.most common.
 Jaw lesions most often in maxilla; reportedJaw lesions most often in maxilla; reported
over a wide age range, average age of 41.6over a wide age range, average age of 41.6
and 20% < age 20.and 20% < age 20.www.indiandentalacademy.com
ChondrosarcomaChondrosarcoma 22
 Most often produces a painless mass; mayMost often produces a painless mass; may
be associated with loosening of teeth,be associated with loosening of teeth,
epistaxis, nasal obstruction and visualepistaxis, nasal obstruction and visual
disturbance.disturbance.
 Lesions grades from I-IV; influence onLesions grades from I-IV; influence on
outcome; most jaw lesion grade I or II.outcome; most jaw lesion grade I or II.
 Size, location and grade influenceSize, location and grade influence
outcome; average 5 year survival of 67.5%outcome; average 5 year survival of 67.5%
with late recurrence & metastasis reducingwith late recurrence & metastasis reducing
long-term survival to less than 44%.long-term survival to less than 44%.www.indiandentalacademy.com
Ewing’s SarcomaEwing’s Sarcoma 11
 Primary malignancy of bone of uncertainPrimary malignancy of bone of uncertain
histogenesis with recent evidence ofhistogenesis with recent evidence of
neuroendocrine origin.neuroendocrine origin.
 85-90% show reciprocal translocation [t(11;22)85-90% show reciprocal translocation [t(11;22)
(q24;q12)].(q24;q12)].
 6-8% of primary malignancies of bone; long6-8% of primary malignancies of bone; long
bones, pelvis and ribs; jaw or craniofacialbones, pelvis and ribs; jaw or craniofacial
lesions are very uncommon (1-2%).lesions are very uncommon (1-2%).
 Peak incidence in second decade (50%); 80%Peak incidence in second decade (50%); 80%
less than age 20; male slightly > females.less than age 20; male slightly > females.
www.indiandentalacademy.com
Ewing’s SarcomaEwing’s Sarcoma 22
 Rare in blacks.Rare in blacks.
 Pain, paresthesia and swelling with looseningPain, paresthesia and swelling with loosening
of teeth.of teeth.
 Ill-defined lytic lesion; “onion-skin” periostealIll-defined lytic lesion; “onion-skin” periosteal
reaction of long bones rare in jaws.reaction of long bones rare in jaws.
 Small round cells; PAS positive diastase labile;Small round cells; PAS positive diastase labile;
angiotropism and necrosis; MIC2 glycoproteinangiotropism and necrosis; MIC2 glycoprotein
detected by immunoperoxidase (CD99).detected by immunoperoxidase (CD99).
 Combined surgery, multidrug chemo &Combined surgery, multidrug chemo &
radiotherapy 5-year survival 40-80; pelvis worst.radiotherapy 5-year survival 40-80; pelvis worst.
www.indiandentalacademy.com
Metastatic Tumors to theMetastatic Tumors to the
JawsJaws 11
 The jaws are regarded as an uncommon siteThe jaws are regarded as an uncommon site
for metastasis although a study of autopsiesfor metastasis although a study of autopsies
revealed 10/62 (16%) carcinomas involved therevealed 10/62 (16%) carcinomas involved the
mandible microscopically though negativemandible microscopically though negative
radiographically.radiographically.
 80% of reported case are in mandible.80% of reported case are in mandible.
 Symptoms include pain, loosening of tooth, aSymptoms include pain, loosening of tooth, a
mass or paresthesia (numb-chin syndrome).mass or paresthesia (numb-chin syndrome).
 Lucent lesion, well or ill definedLucent lesion, well or ill defined (“moth-eaten”).(“moth-eaten”).
www.indiandentalacademy.com
Metastatic Tumors to theMetastatic Tumors to the
JawsJaws 22
 Involvement of alveolus may mimicInvolvement of alveolus may mimic
periodontal or periapical disease; occasionalperiodontal or periapical disease; occasional
widened periodontal ligament space noted.widened periodontal ligament space noted.
 Metastatic carcinomas (especially breast &Metastatic carcinomas (especially breast &
prostate) may stimulate new bone formationprostate) may stimulate new bone formation
with a resultant radiopaque or mixed lesion.with a resultant radiopaque or mixed lesion.
 Most common sites of primary are breast,Most common sites of primary are breast,
lung, thyroid, prostate & kidney.lung, thyroid, prostate & kidney.
 Stage IV primary (not oral cancer) with aStage IV primary (not oral cancer) with a
usually survival of less than one year.usually survival of less than one year.
www.indiandentalacademy.com

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Bone pathology/ dental crown & bridge courses

  • 1. BONE PATHOLOGYBONE PATHOLOGY INDIAN DENTALACADEMYINDIAN DENTALACADEMY Leader in continuing Dental EducationLeader in continuing Dental Education www.indiandentalacademy.com
  • 2. Osteogenesis ImperfectaOsteogenesis Imperfecta 11  A heterogeneous group of heritableA heterogeneous group of heritable disorders characterized by impairment ofdisorders characterized by impairment of collagen maturation.collagen maturation.  Mutation of COL1A1 (chromosome 17) orMutation of COL1A1 (chromosome 17) or COL1A2 (7).COL1A2 (7).  Most common type of heritable boneMost common type of heritable bone disease (1/8000).disease (1/8000). www.indiandentalacademy.com
  • 3. Osteogenesis ImperfectaOsteogenesis Imperfecta 22  Main features are bone fragility, blueMain features are bone fragility, blue sclera, altered teeth, hypoacusis (hearingsclera, altered teeth, hypoacusis (hearing loss) long bone and spine deformity andloss) long bone and spine deformity and joint hyperextensibility.joint hyperextensibility.  Oral findings include dentinogenesisOral findings include dentinogenesis imperfecta, maxillary hypoplasia withimperfecta, maxillary hypoplasia with class III malocclusion and radiographicclass III malocclusion and radiographic lesions similar to florid cemento-osseouslesions similar to florid cemento-osseous dysplasia.dysplasia. www.indiandentalacademy.com
  • 4. www.indiandentalacademy.com Indian Dental academy • www.indiandentalacademy.com • Leader continuing dental education • Offer both online and offline dental courses
  • 5. Osteogenesis ImperfectaOsteogenesis Imperfecta 33  Type I: AD; Most common and mildestType I: AD; Most common and mildest form, 10% congenital fracture; 10% neverform, 10% congenital fracture; 10% never fracture; blue sclera; hearing loss beforefracture; blue sclera; hearing loss before 30; hyperextensibility and tooth defects30; hyperextensibility and tooth defects variable.variable.  Type II: AR/AD; Most severe form; 90%Type II: AR/AD; Most severe form; 90% stillborn or die before 4 weeks; bluestillborn or die before 4 weeks; blue sclera; opalescent teeth may be present.sclera; opalescent teeth may be present. www.indiandentalacademy.com
  • 6. Osteogenesis ImperfectaOsteogenesis Imperfecta 44  Type III: AD/AR: Moderate to severe boneType III: AD/AR: Moderate to severe bone fragility; blue sclera fades over time;fragility; blue sclera fades over time; hyperextensibility and hearing loss common;hyperextensibility and hearing loss common; 2/3 die in childhood from cardiopulmonary2/3 die in childhood from cardiopulmonary failure 2failure 2º to kyphoscoliosis; some haveº to kyphoscoliosis; some have opalescent dentin.opalescent dentin.  Type IV: AD; Mild to moderate bone fragility-Type IV: AD; Mild to moderate bone fragility- 50% congenital fractures with frequency50% congenital fractures with frequency decreasing with time; blue sclera fades overdecreasing with time; blue sclera fades over time; opalescent dentin variable.time; opalescent dentin variable. www.indiandentalacademy.com
  • 7. OsteopetrosisOsteopetrosis 11 (Albers-Sch(Albers-Schönberg Disease)önberg Disease)  A group of rare hereditary skeletalA group of rare hereditary skeletal defects of increased bone densitydefects of increased bone density resulting from defective remodeling dueresulting from defective remodeling due to failure in osteoclast function.to failure in osteoclast function.  Endochondral, endosteal and periostealEndochondral, endosteal and periosteal growth without concomitant resorptiongrowth without concomitant resorption results in thickening of bone.results in thickening of bone.  Incidence estimated between 1 inIncidence estimated between 1 in 100,000-500,000.100,000-500,000. www.indiandentalacademy.com
  • 8. OsteopetrosisOsteopetrosis 22 (Albers-Sch(Albers-Schönberg Disease)önberg Disease)  Infantile (malignant form): AR; marrowInfantile (malignant form): AR; marrow failure, fracture and cranial nervefailure, fracture and cranial nerve compression (blindness, deafness,compression (blindness, deafness, facial paralysis.facial paralysis.  Hepatosplenomegaly, infections andHepatosplenomegaly, infections and osteomyelitis.osteomyelitis.  Delayed tooth eruption; extractionDelayed tooth eruption; extraction osteomyelitis .osteomyelitis . www.indiandentalacademy.com
  • 9. OsteopetrosisOsteopetrosis 33 (Albers-Sch(Albers-Schönberg Disease)önberg Disease)  Adult (benign form): AD; limited mostly to axialAdult (benign form): AD; limited mostly to axial skeleton; bone pain is common but nearly 40%skeleton; bone pain is common but nearly 40% are asymptomatic; marrow failure rare.are asymptomatic; marrow failure rare.  Variants with cranial nerve compression-withoutVariants with cranial nerve compression-without fracture or fracture without cranial nervefracture or fracture without cranial nerve compression .compression .  Treatment of infantile with BMT for cure;Treatment of infantile with BMT for cure; interferon, calcitriol, steroids, PTH, MCSF,interferon, calcitriol, steroids, PTH, MCSF, erythropoietin.erythropoietin.  Supportive measures; transfusion, antibiotics,Supportive measures; transfusion, antibiotics, surgery & hyperbaric oxygen.surgery & hyperbaric oxygen.www.indiandentalacademy.com
  • 10. Cleidocranial DysplasiaCleidocranial Dysplasia 11  Defect in CBFA1 gene (6p21); AD with 40%Defect in CBFA1 gene (6p21); AD with 40% sporadic cases; defect in osteoblasticsporadic cases; defect in osteoblastic differentiation and bone formation.differentiation and bone formation.  Clavicles show hypoplasia or malformation;Clavicles show hypoplasia or malformation; about 10% one/both clavicles are absent.about 10% one/both clavicles are absent.  Short stature, large head with frontal & parietalShort stature, large head with frontal & parietal bossing, hypertelorism, broad nose, depressedbossing, hypertelorism, broad nose, depressed nasal bridge.nasal bridge.  Persistent open fontanels and cranial sutures.Persistent open fontanels and cranial sutures. www.indiandentalacademy.com
  • 11. Cleidocranial DysplasiaCleidocranial Dysplasia 22  Dental & gnathic manifestations includeDental & gnathic manifestations include narrow, high-arched palate w/o cleft,narrow, high-arched palate w/o cleft, prolonged retention of deciduous teeth,prolonged retention of deciduous teeth, delayed eruption of permanent teeth,delayed eruption of permanent teeth, numerous unerupted permanent andnumerous unerupted permanent and supernumerary teeth (lack 2supernumerary teeth (lack 2º cementum)º cementum).. Narrow ramus, pointed coronoid processes,Narrow ramus, pointed coronoid processes, thin zygomatic arch, small/absent maxillarythin zygomatic arch, small/absent maxillary sinuses, acute gonial angle, prognathismsinuses, acute gonial angle, prognathism.. www.indiandentalacademy.com
  • 12. Focal OsteoporoticFocal Osteoporotic Marrow DefectMarrow Defect  Non-pathologic area of hematopoietic marrowNon-pathologic area of hematopoietic marrow sufficient in size to appear radiographically assufficient in size to appear radiographically as a radiolucency.a radiolucency.  Theories as to cause include aberrant boneTheories as to cause include aberrant bone regeneration following tooth extraction,regeneration following tooth extraction, persistent fetal marrow or marrow hypoplasiapersistent fetal marrow or marrow hypoplasia due to increased demand for erythrocytes.due to increased demand for erythrocytes.  Asymptomatic ill-defined circumscribedAsymptomatic ill-defined circumscribed lucency from a few to several cm in size.lucency from a few to several cm in size.  More than 75% of cases in adult females in anMore than 75% of cases in adult females in an edentulous area posterior mandible; biopsy.edentulous area posterior mandible; biopsy.www.indiandentalacademy.com
  • 13. IdiopathicIdiopathic OsteosclerosisOsteosclerosis 11  Focal area of increased radiodensity ofFocal area of increased radiodensity of unknown cause.unknown cause.  Also known as dense bone island, boneAlso known as dense bone island, bone eburnation, bone whorl, bone scar, enostosis,eburnation, bone whorl, bone scar, enostosis, focal (periapical) osteopetrosis.focal (periapical) osteopetrosis.  Peak age 20’s; no sex predilection.Peak age 20’s; no sex predilection.  Well defined rounded or elliptic radiodenseWell defined rounded or elliptic radiodense mass 3-20 mm.mass 3-20 mm.  Common (5%) in mandible (90%); first molar, 2Common (5%) in mandible (90%); first molar, 2ndnd premolar or 2premolar or 2ndnd molar; most are periapical.molar; most are periapical. www.indiandentalacademy.com
  • 14. IdiopathicIdiopathic OsteosclerosisOsteosclerosis 22  Need to rule-out condensing osteitis (pulpNeed to rule-out condensing osteitis (pulp disease).disease).  Diagnosis based on history clinical andDiagnosis based on history clinical and radiographic features; pre-existingradiographic features; pre-existing radiographs and periodic reassessment.radiographs and periodic reassessment.  Biopsy if expansion is present.Biopsy if expansion is present.  Because lesion becomes stable and indolent,Because lesion becomes stable and indolent, not treatment necessary.not treatment necessary. www.indiandentalacademy.com
  • 15. Massive OsteolysisMassive Osteolysis 11  Also known as Gorham disease, Gorham-Also known as Gorham disease, Gorham- Stout syndrome, vanishing bone disease andStout syndrome, vanishing bone disease and phantom bone disease.phantom bone disease.  Rare, idiopathic spontaneous progressiveRare, idiopathic spontaneous progressive destruction of one or more bones; maydestruction of one or more bones; may represent hamangiomatosis of bone.represent hamangiomatosis of bone.  Most often in children & young adults; 50%Most often in children & young adults; 50% report antecedent trauma.report antecedent trauma.  Pelvis, humeral head or shaft & axial skeletonPelvis, humeral head or shaft & axial skeleton most common sites.most common sites. www.indiandentalacademy.com
  • 16. Massive OsteolysisMassive Osteolysis 22  30% maxillofacial involvement, usually30% maxillofacial involvement, usually the mandible.the mandible.  Signs include mobile teeth, pain,Signs include mobile teeth, pain, malocclusion, midline deviation,malocclusion, midline deviation, pathologic fracture and obstructive sleeppathologic fracture and obstructive sleep apnea.apnea.  Intramedullary lucency, loss of laminaIntramedullary lucency, loss of lamina dura, thinning of cortex.dura, thinning of cortex.  Spontaneous arrest; radiation/surgery.Spontaneous arrest; radiation/surgery.www.indiandentalacademy.com
  • 17. Paget’s Disease of BonePaget’s Disease of Bone 11  Also known as osteitis deformansAlso known as osteitis deformans  Idiopathic disorder of abnormal anarchicIdiopathic disorder of abnormal anarchic resorption and deposition of boneresorption and deposition of bone (abnormal remodeling).(abnormal remodeling).  Relatively common after age 40; 1/100-Relatively common after age 40; 1/100- 150 (increasing to 1/10 by age 90); M>F;150 (increasing to 1/10 by age 90); M>F; white > blacks or Asians.white > blacks or Asians.  Usually polyostotic (multiple boneUsually polyostotic (multiple bone affected), rarely monostotic (single bone)affected), rarely monostotic (single bone)www.indiandentalacademy.com
  • 18. Paget’s Disease of BonePaget’s Disease of Bone 22  Lumbar vertebrae, pelvis, skull & femur; jawLumbar vertebrae, pelvis, skull & femur; jaw involvement in 17%, usually the maxilla.involvement in 17%, usually the maxilla.  Bowing of legs, leontiasis ossea, fracture,Bowing of legs, leontiasis ossea, fracture, bone and joint pain, pressure neuropathy.bone and joint pain, pressure neuropathy.  May cause progressive alveolar ridgeMay cause progressive alveolar ridge expansion, spacing of teeth and “dentalexpansion, spacing of teeth and “dental appliances that no longer fit the mouth”.appliances that no longer fit the mouth”.  Altered trabecular pattern initially fromAltered trabecular pattern initially from decreased bone density evolves to patchydecreased bone density evolves to patchy sclerotic areas having a “cotton wool”sclerotic areas having a “cotton wool” appearance.appearance. www.indiandentalacademy.com
  • 19. Paget’s Disease of BonePaget’s Disease of Bone 33  Alternating resorption and formation of boneAlternating resorption and formation of bone produces a characteristic “mosaic”pattern andproduces a characteristic “mosaic”pattern and marrow replaced by highly vascular fibrousmarrow replaced by highly vascular fibrous connective tissue.connective tissue.  Elevation of serum alkaline phosphataseElevation of serum alkaline phosphatase treated if elevated by more than 25-50%;treated if elevated by more than 25-50%; elevated urinary hydroxyproline levels.elevated urinary hydroxyproline levels.  Increased risk of osteosarcoma (1%) and giantIncreased risk of osteosarcoma (1%) and giant cell tumors of bone.cell tumors of bone.  Treatment with calcitonin & biophosphonate;Treatment with calcitonin & biophosphonate; plicamycin for refractory casesplicamycin for refractory caseswww.indiandentalacademy.com
  • 20. Central Giant CellCentral Giant Cell Granuloma (Lesion)Granuloma (Lesion) 11  A non-neoplastic lesion of the jaws; mostA non-neoplastic lesion of the jaws; most common before age 30 (60%); females>males;common before age 30 (60%); females>males; 70% in mandible, especially anterior two-thirds70% in mandible, especially anterior two-thirds  Most often asymptomatic or produce painlessMost often asymptomatic or produce painless expansion; occasionally pain, paresthesia orexpansion; occasionally pain, paresthesia or cortical perforation.cortical perforation.  Non-aggressive lesions are most common,Non-aggressive lesions are most common, produce few symptoms and are slow growing.produce few symptoms and are slow growing.  Aggressive lesions produce pain, rapidAggressive lesions produce pain, rapid growth, cortical perforation and rootgrowth, cortical perforation and root resorption.resorption. www.indiandentalacademy.com
  • 21. Central Giant CellCentral Giant Cell Granuloma (Lesion)Granuloma (Lesion) 22  Radiographic lesions range from less than 5Radiographic lesions range from less than 5 mm to greater than 10 cm; unilocular ormm to greater than 10 cm; unilocular or multilocular; differential diagnosis required.multilocular; differential diagnosis required.  Histopathologically consist ofHistopathologically consist of multinucleated giant cells in a stroma ofmultinucleated giant cells in a stroma of spindle-shaped mesenchymal cells usuallyspindle-shaped mesenchymal cells usually with numerous small vessel and hemorrhagewith numerous small vessel and hemorrhage —indistinguishable from—indistinguishable from hyperparathyroidism, cherubism andhyperparathyroidism, cherubism and aneurysmal bone cyst.aneurysmal bone cyst. www.indiandentalacademy.com
  • 22. Central Giant CellCentral Giant Cell Granuloma (Lesion)Granuloma (Lesion) 33  R/O other causes for “giant cell lesions”R/O other causes for “giant cell lesions”  Treatment by thorough curettage;Treatment by thorough curettage; recurrence rate 15-20%; resection forrecurrence rate 15-20%; resection for larger lesions.larger lesions.  Investigational treatments of repeatedInvestigational treatments of repeated intrabony lesional injection ofintrabony lesional injection of triamcinolone or daily calcitonintriamcinolone or daily calcitonin injection (or nasal spray) for one year asinjection (or nasal spray) for one year as alternatives to deforming surgery.alternatives to deforming surgery. www.indiandentalacademy.com
  • 23. Giant Cell TumorGiant Cell Tumor  Considered a true neoplasm usuallyConsidered a true neoplasm usually found in long bones.found in long bones.  Although it can be histopathologicallyAlthough it can be histopathologically indistinguishable from central giant cellindistinguishable from central giant cell granuloma, the biologic behavior of GCTgranuloma, the biologic behavior of GCT different from that of CGCG; has adifferent from that of CGCG; has a higher recurrence rate and 10% rate ofhigher recurrence rate and 10% rate of metastasize.metastasize. www.indiandentalacademy.com
  • 24. CherubismCherubism 11  Rare development condition of the jaws, generallyRare development condition of the jaws, generally AD (4p16) with high penetrance but variableAD (4p16) with high penetrance but variable expressivity.expressivity.  Onset usually 2-5; progresses until puberty,Onset usually 2-5; progresses until puberty, becomes stable, then slowly regresses.becomes stable, then slowly regresses.  Mandibular involvement of posterior areas resultsMandibular involvement of posterior areas results in bilateral symmetrical multilocular expansion;in bilateral symmetrical multilocular expansion; maxillary involvement usually in tuberosity areas.maxillary involvement usually in tuberosity areas.  Involved bone produces widening and distortion ofInvolved bone produces widening and distortion of alveolar ridges, tooth displacement, alteredalveolar ridges, tooth displacement, altered eruption, impaired mastication & speecheruption, impaired mastication & speech difficulties.difficulties. www.indiandentalacademy.com
  • 25. CherubismCherubism 22  Clavicle, ribs and humerus reported as rareClavicle, ribs and humerus reported as rare extragnathic sites.extragnathic sites.  Microscopically similar to CGCG;Microscopically similar to CGCG; eosinophilic cuff-like deposits around smalleosinophilic cuff-like deposits around small vessels a helpful finding. Long-standingvessels a helpful finding. Long-standing lesions become more fibrous with fewerlesions become more fibrous with fewer giant cells.giant cells.  Prognosis variable; return to normalcy forPrognosis variable; return to normalcy for many; some with significant deformity;many; some with significant deformity; dental management sometimes difficult.dental management sometimes difficult. www.indiandentalacademy.com
  • 26. Simple Bone CystSimple Bone Cyst 11  Also known as traumatic bone cyst,Also known as traumatic bone cyst, hemorrhagic bone cyst, solitary bonehemorrhagic bone cyst, solitary bone cyst & idiopathic bone cavity.cyst & idiopathic bone cavity.  A benign, empty or fluid-filled cavityA benign, empty or fluid-filled cavity within bone devoid of epithelial lining.within bone devoid of epithelial lining.  Trauma might play role in some cases butTrauma might play role in some cases but other theories are offered.other theories are offered.  Majority occur in long bones, ages 10-20;Majority occur in long bones, ages 10-20; mandible; 60% of jaw lesions in males.mandible; 60% of jaw lesions in males.www.indiandentalacademy.com
  • 27. Simple Bone CystSimple Bone Cyst 22  Usually asymptomatic; 20% have swelling ofUsually asymptomatic; 20% have swelling of jaw.jaw.  Well-delineated radiolucency; usually inWell-delineated radiolucency; usually in premolar-molar region, often scalloped upwardpremolar-molar region, often scalloped upward between the roots of teeth; rarely multilocular.between the roots of teeth; rarely multilocular.  Pulp test teeth; can be associated withPulp test teeth; can be associated with cemento-osseous dysplasia.cemento-osseous dysplasia.  Diagnosis based on clinical, radiographic andDiagnosis based on clinical, radiographic and surgical findings.surgical findings.  Gnathic lesions treated by surgical explorationGnathic lesions treated by surgical exploration and follow-up.and follow-up.www.indiandentalacademy.com
  • 28. Aneurysmal Bone CystAneurysmal Bone Cyst 11  A non-neoplastic lesion of uncertain etiologyA non-neoplastic lesion of uncertain etiology that may mimic the growth and behavior of athat may mimic the growth and behavior of a neoplasm of bone. It is not a “true cyst.”neoplasm of bone. It is not a “true cyst.”  Trauma, vascular malformation or anTrauma, vascular malformation or an associated neoplasm that disrupts the normalassociated neoplasm that disrupts the normal hemodynamics of bone have been proposedhemodynamics of bone have been proposed as causes.as causes.  An intraosseous accumulation of variable-An intraosseous accumulation of variable- sized blood-filled spaces surrounded bysized blood-filled spaces surrounded by cellular fibrous CT & reactive bone.cellular fibrous CT & reactive bone. www.indiandentalacademy.com
  • 29. Aneurysmal Bone CystAneurysmal Bone Cyst 22  Most common in shaft of long bone; ageMost common in shaft of long bone; age <30; gnathic lesions account for 2% with a<30; gnathic lesions account for 2% with a mean age of 20 years; most often inmean age of 20 years; most often in posterior mandible.posterior mandible.  Rapid swelling, pain often reported.Rapid swelling, pain often reported.  Unilocular or multilocular radiolucencyUnilocular or multilocular radiolucency with cortical expansion; ballooning “blow-with cortical expansion; ballooning “blow- out” distension of bone contour;out” distension of bone contour; sometimes radiopaque foci or smallsometimes radiopaque foci or small trabaculae seen.trabaculae seen.www.indiandentalacademy.com
  • 30. Aneurysmal Bone CystAneurysmal Bone Cyst 33  At surgery lesion often describe as aAt surgery lesion often describe as a “blood-soaked sponge”.“blood-soaked sponge”.  20% of jaw lesions associated with other20% of jaw lesions associated with other pathosis (fibro-osseous lesion of CGCG).pathosis (fibro-osseous lesion of CGCG).  Curettage or enucleation; recurrenceCurettage or enucleation; recurrence variable (8-60%); resection rarelyvariable (8-60%); resection rarely needed.needed. www.indiandentalacademy.com
  • 31. Fibro-osseous LesionsFibro-osseous Lesions of the Jawsof the Jaws Fibrous dysplasiaFibrous dysplasia Cemento-osseous dysplasiaCemento-osseous dysplasia a.a. Focal cemento-osseous dysplasiaFocal cemento-osseous dysplasia b.b. Periapical cemento-osseous dysplasiaPeriapical cemento-osseous dysplasia c.c. Florid cemento-osseous dysplasiaFlorid cemento-osseous dysplasia •Ossifying fibromaOssifying fibroma www.indiandentalacademy.com
  • 32. Fibrous DysplasiaFibrous Dysplasia 11  Developmental condition of replacement ofDevelopmental condition of replacement of normal bone by an excessive proliferation ofnormal bone by an excessive proliferation of cellular fibrous connective tissue intermixedcellular fibrous connective tissue intermixed with irregular bone trabeculae.with irregular bone trabeculae.  Sporadic condition attributed to mutation ofSporadic condition attributed to mutation of GNAS 1 gene (guanine nucleotide-binding,GNAS 1 gene (guanine nucleotide-binding, -- stimulating activity peptide); clinical severitystimulating activity peptide); clinical severity determined by time when the mutation occurs;determined by time when the mutation occurs; early embryonic mutation produces polyostoticearly embryonic mutation produces polyostotic disease with other abnormalities; postnataldisease with other abnormalities; postnatal monostotic disease.monostotic disease. www.indiandentalacademy.com
  • 33. Fibrous DysplasiaFibrous Dysplasia 22  Monostotic accounts for 80-85% of cases; jawsMonostotic accounts for 80-85% of cases; jaws are common location; maxilla > mandible;are common location; maxilla > mandible; maxillary lesions sometimes part of widermaxillary lesions sometimes part of wider craniofacial fibrous dysplasia.craniofacial fibrous dysplasia.  Radiographic poorly demarcated “ground-Radiographic poorly demarcated “ground- glass” appearance of affected bone causingglass” appearance of affected bone causing expansion; in long bones often radiolucent,expansion; in long bones often radiolucent, multilocular & expansile.multilocular & expansile.  PDL may be narrow and lamina dura indistinct;PDL may be narrow and lamina dura indistinct; inferior alveolar nerve canal displacedinferior alveolar nerve canal displaced superiorly.superiorly. www.indiandentalacademy.com
  • 34. Fibrous DysplasiaFibrous Dysplasia 33  Polyostotic (PFD) involves two or morePolyostotic (PFD) involves two or more bones.bones.  Jaffe-Lichtenstein syndrome consists ofJaffe-Lichtenstein syndrome consists of PFD withPFD with café au laitcafé au lait pigmentation.pigmentation.  McCune-Albright syndrome consists ofMcCune-Albright syndrome consists of PFD,PFD, café au laitcafé au lait pigmentation andpigmentation and multiple endocrinopathies such a sexualmultiple endocrinopathies such a sexual precocity, pituitary adenoma orprecocity, pituitary adenoma or hyperthyroidism.hyperthyroidism. www.indiandentalacademy.com
  • 35. Fibrous DysplasiaFibrous Dysplasia 44  Histopathologically consists ofHistopathologically consists of trabeculae of woven bone in a cellular,trabeculae of woven bone in a cellular, fibrous stroma (Chinese characters).fibrous stroma (Chinese characters).  Excision or resection of small jawExcision or resection of small jaw lesions; extensive or diffuse lesions maylesions; extensive or diffuse lesions may require repeated recontouring forrequire repeated recontouring for cosmesis; 25-50% regrowth after shave-cosmesis; 25-50% regrowth after shave- down.down.  Occasional development ofOccasional development of osteosarcoma in bone affected by FD.osteosarcoma in bone affected by FD.www.indiandentalacademy.com
  • 36. Cemento-OsseousCemento-Osseous DysplasiasDysplasias 11  Non-neoplastic fibro-osseous alteration ofNon-neoplastic fibro-osseous alteration of bone in the tooth bearing areas of the jaws.bone in the tooth bearing areas of the jaws.  Three distinct radiographic/clinical patternsThree distinct radiographic/clinical patterns that share a common histopathologicthat share a common histopathologic appearance.appearance.  Cellular connective tissue containing aCellular connective tissue containing a mixture of woven bone, lamellar bone andmixture of woven bone, lamellar bone and cementum-like particles; ratio of fibrouscementum-like particles; ratio of fibrous tissue decreases over time as it becomestissue decreases over time as it becomes sclerotic by fusion of calcified elements.sclerotic by fusion of calcified elements. www.indiandentalacademy.com
  • 37. Cemento-OsseousCemento-Osseous DysplasiasDysplasias (Focal)(Focal)22  Focal cemento-osseous dysplasiaFocal cemento-osseous dysplasia occursoccurs most frequently in young to middle aged whitemost frequently in young to middle aged white females (9:1,F:M).females (9:1,F:M).  Solitary radiolucent to radiopaque lesion, lessSolitary radiolucent to radiopaque lesion, less than 1.5 cm, if opaque with thin radiolucentthan 1.5 cm, if opaque with thin radiolucent rim; most often in posterior mandible; well-rim; most often in posterior mandible; well- defined with slight irregular borders.defined with slight irregular borders.  Easily fragmented gritty tissue that can beEasily fragmented gritty tissue that can be curetted but does not separate easily from thecuretted but does not separate easily from the surrounding bone.surrounding bone.  Monitor once diagnosis established by biopsy.Monitor once diagnosis established by biopsy.www.indiandentalacademy.com
  • 38. Cemento-OsseousCemento-Osseous Dysplasias (Periapical)Dysplasias (Periapical) 33  Periapical cemento-osseous dysplasiaPeriapical cemento-osseous dysplasia occursoccurs most frequently in black females (70%) 30-50most frequently in black females (70%) 30-50 y.o.y.o.  F:M, 14:1.F:M, 14:1.  Asymptomatic, anterior mandible, typicallyAsymptomatic, anterior mandible, typically multifocal, periapical to vital teeth. Must pulpmultifocal, periapical to vital teeth. Must pulp test teeth.test teeth.  Lucent > opaque over time; < 1cm; PDL intact,Lucent > opaque over time; < 1cm; PDL intact, opacity not fused to tooth.opacity not fused to tooth.  No treatment necessary.No treatment necessary. www.indiandentalacademy.com
  • 39. Cemento-OsseousCemento-Osseous Dysplasias (Florid)Dysplasias (Florid) 33  Florid cemento-osseous dysplasiaFlorid cemento-osseous dysplasia occurs mostoccurs most frequently in middle aged to older black females (90%).frequently in middle aged to older black females (90%).  Multifocal involvement but not limited to the anteriorMultifocal involvement but not limited to the anterior mandible; often bilaterally symmetric.mandible; often bilaterally symmetric.  Most often asymptomatic; pain, swelling or signs ofMost often asymptomatic; pain, swelling or signs of osteomyelitis.osteomyelitis.  Lucent/opaque; sometimes associated with simpleLucent/opaque; sometimes associated with simple bone cyst.bone cyst.  No treatment for asymptomatic lesions; exploration ofNo treatment for asymptomatic lesions; exploration of simple bone cysts; avoid surgical exposure of affectedsimple bone cysts; avoid surgical exposure of affected sclerotic areas; symptomatic areas are treated thesclerotic areas; symptomatic areas are treated the same as for osteomyelitis.same as for osteomyelitis. www.indiandentalacademy.com
  • 40. Familial GigantiformFamilial Gigantiform CementomaCementoma  Uncommon hereditary AD disorder of gnathicUncommon hereditary AD disorder of gnathic bone in which massive sclerotic masses ofbone in which massive sclerotic masses of disorganized mineralized tissue develop.disorganized mineralized tissue develop.  Begin as multiple periapical lucencies whichBegin as multiple periapical lucencies which expand to produce a mixed radiolucent-expand to produce a mixed radiolucent- radiopaque pattern.radiopaque pattern.  Anemia and multiple polypoid adenomas of theAnemia and multiple polypoid adenomas of the uterus reported in some affected females.uterus reported in some affected females.  Extensive resection & reconstruction may beExtensive resection & reconstruction may be needed to achieve acceptable cosmesis.needed to achieve acceptable cosmesis. www.indiandentalacademy.com
  • 41. Ossifying FibromaOssifying Fibroma  AA true neoplasm of an admixture of fibrous tissue &true neoplasm of an admixture of fibrous tissue & bony trabeculae, cementum-like spherules, or both.bony trabeculae, cementum-like spherules, or both.  Most frequent in third-fourth decade; femaleMost frequent in third-fourth decade; female predilection.predilection.  Premolar-molar region of mandible most frequent site.Premolar-molar region of mandible most frequent site.  Small lesions asymptomatic; larger lesions produceSmall lesions asymptomatic; larger lesions produce painless swelling.painless swelling.  Well defined radiolucency, often with a scleroticWell defined radiolucency, often with a sclerotic border, containing varying amounts of opacity. Largerborder, containing varying amounts of opacity. Larger lesions produce downward bow of the inferior cortexlesions produce downward bow of the inferior cortex of mandible.of mandible.  Enucleation; resection for large lesions.Enucleation; resection for large lesions.www.indiandentalacademy.com
  • 42. Juvenile OssifyingJuvenile Ossifying FibromaFibroma 11  Differs from common OF by ageDiffers from common OF by age (younger), location (maxilla) & biologic(younger), location (maxilla) & biologic behavior (sometime aggressive and/orbehavior (sometime aggressive and/or recurrent).recurrent).  Two microscopic types; trabecular &Two microscopic types; trabecular & psammomatoid; age at diagnosis 11 andpsammomatoid; age at diagnosis 11 and 22 respectively.22 respectively.  Maxilla often with impingement onMaxilla often with impingement on sinuses, orbit, nose and cranium.sinuses, orbit, nose and cranium. www.indiandentalacademy.com
  • 43. Juvenile OssifyingJuvenile Ossifying FibromaFibroma 22  Richly cellular fibrous tissue showingRichly cellular fibrous tissue showing strand of osteoid or psammomatoidstrand of osteoid or psammomatoid ossicles.ossicles.  Growth of individual tumors varies fromGrowth of individual tumors varies from slow to rapid; tends to be rapid inslow to rapid; tends to be rapid in younger patients.younger patients.  Complete curettage for small lesions;Complete curettage for small lesions; resection for larger, aggressive lesions;resection for larger, aggressive lesions; recurrence 30-58%.recurrence 30-58%. www.indiandentalacademy.com
  • 44. OsteomaOsteoma  Benign tumors (neoplasm?) of mature compact orBenign tumors (neoplasm?) of mature compact or cancellous bone that are restricted to the craniofacialcancellous bone that are restricted to the craniofacial skeleton.skeleton.  May be periosteal or endoseal; perioseal lesionsMay be periosteal or endoseal; perioseal lesions produce slowing growing mass; endosteal lesionsproduce slowing growing mass; endosteal lesions asymptomatic or slow enlargement.asymptomatic or slow enlargement.  Condylar lesion produces lobulated mass; deviation ofCondylar lesion produces lobulated mass; deviation of midline.midline.  Radiographically a circumscribed sclerotic massRadiographically a circumscribed sclerotic mass  Symptomatic or deforming lesions removed bySymptomatic or deforming lesions removed by conservative excision.conservative excision. www.indiandentalacademy.com
  • 45. Gardner SyndromeGardner Syndrome  Rare inherited AD disorder (gene on chromosome 5)Rare inherited AD disorder (gene on chromosome 5)  Within the spectrum of familiar colorectal polyposisWithin the spectrum of familiar colorectal polyposis  1 in 8,300-16,0001 in 8,300-16,000  Osteomas of any bone, skull, paranasal sinuses andOsteomas of any bone, skull, paranasal sinuses and mandible common.mandible common.  Supernumerary teeth in 20%Supernumerary teeth in 20%  Epidermoid cysts of skin, desmoid tumor of skin,Epidermoid cysts of skin, desmoid tumor of skin, adenomatous colonic polyps > colon cancer,adenomatous colonic polyps > colon cancer, pigmented lesions of ocular fundus, 100-fold increasepigmented lesions of ocular fundus, 100-fold increase in thyroid cancer in females.in thyroid cancer in females.  Colonectomy; elective prophylactic thyroidectomy,Colonectomy; elective prophylactic thyroidectomy, elective removal of osteomas; cysts and other tumors.elective removal of osteomas; cysts and other tumors.www.indiandentalacademy.com
  • 46. Osteoblastoma &Osteoblastoma & Osteoid OsteomaOsteoid Osteoma  Rare neoplasms of bone (<1%) are microscopicallyRare neoplasms of bone (<1%) are microscopically identical; osteoblastomas most frequent in vertebralidentical; osteoblastomas most frequent in vertebral column, sacrum, calvarium, long bones small bones ofcolumn, sacrum, calvarium, long bones small bones of hands/feet; osteoid osteoma most often femur, tibia &hands/feet; osteoid osteoma most often femur, tibia & phalanges.phalanges.  Osteoid osteoma produce prostaglandin > pain relievedOsteoid osteoma produce prostaglandin > pain relieved by aspirin; not osteoblastoma.by aspirin; not osteoblastoma.  In jaws, slight mandible predilection in posterior area;In jaws, slight mandible predilection in posterior area; 85% < age 30.85% < age 30.  Painful radiolucent/opaque lesion often 2-4 cm.Painful radiolucent/opaque lesion often 2-4 cm.  Local excision or curettage; 50% chance for recurrenceLocal excision or curettage; 50% chance for recurrence of aggressive lesions.of aggressive lesions.www.indiandentalacademy.com
  • 47. CementoblastomaCementoblastoma  Rare neoplasm thought by some to be identical withRare neoplasm thought by some to be identical with osteoblastoma differing only by its attachment to theosteoblastoma differing only by its attachment to the root of a tooth. <1% of odontogenic tumor.root of a tooth. <1% of odontogenic tumor.  75% in mandible; 90% molar or premolar with 50%75% in mandible; 90% molar or premolar with 50% involving first molar.involving first molar.  Children and young adults; 50% <age 20 & 75% < ageChildren and young adults; 50% <age 20 & 75% < age 30.30.  Pain reported by 2/3Pain reported by 2/3  Opaque mass fused to root surrounded by thinOpaque mass fused to root surrounded by thin radiolucent rim.radiolucent rim.  Extraction/removal of mass; removal of mass, rootExtraction/removal of mass; removal of mass, root amputation & endodontics.amputation & endodontics.www.indiandentalacademy.com
  • 48. ChondromaChondroma  Benign neoplasm of hyaline cartilage.Benign neoplasm of hyaline cartilage.  Most common in the short tubular bonesMost common in the short tubular bones of the hands and feet; multiple lesion inof the hands and feet; multiple lesion in Ollier disease or Maffucci syndrome; jawOllier disease or Maffucci syndrome; jaw lesions are uncommon and most are inlesions are uncommon and most are in condyle or anterior maxilla.condyle or anterior maxilla.  Radiolucent with central area of opacityRadiolucent with central area of opacity  Surgical removal; microscopic distinctionSurgical removal; microscopic distinction from low-grade chondrosarcoma difficult.from low-grade chondrosarcoma difficult.www.indiandentalacademy.com
  • 49. Chondromyxoid FibromaChondromyxoid Fibroma  Uncommon benign neoplasm (<1%) most oftenUncommon benign neoplasm (<1%) most often involving metaphysis of long bones; jawinvolving metaphysis of long bones; jaw lesions are rare.lesions are rare.  For jaw lesions average age 30; 75% inFor jaw lesions average age 30; 75% in mandible; pain and swelling most commonmandible; pain and swelling most common symptoms.symptoms.  Circumscribed radiolucency with sclerotic orCircumscribed radiolucency with sclerotic or scalloped margin; size range 1-6.5 cmscalloped margin; size range 1-6.5 cm  Can be confused with chondrosarcomaCan be confused with chondrosarcoma histologically.histologically.  Curettage (25% recur) or surgical resection.Curettage (25% recur) or surgical resection.www.indiandentalacademy.com
  • 50. SynovialSynovial ChondromatosisChondromatosis  Rare benign, non-neoplastic arthropathy ofRare benign, non-neoplastic arthropathy of unknown cause; cartilaginous nodules formunknown cause; cartilaginous nodules form within the synovial membrane.within the synovial membrane.  Most often affects a single large joint; knee,Most often affects a single large joint; knee, elbow, hip and shoulder most common;elbow, hip and shoulder most common; mandible is rare site.mandible is rare site.  Radiographically “loose bodies” in jointRadiographically “loose bodies” in joint appear as round irregular radiopacities;appear as round irregular radiopacities; widened joint space, irregular condylar headwidened joint space, irregular condylar head  Removal of loose bodies; total synovectomy;Removal of loose bodies; total synovectomy; menisectomy.menisectomy.www.indiandentalacademy.com
  • 51. Desmoplastic FibromaDesmoplastic Fibroma  Uncommon bone tumor of fibroblastic tissueUncommon bone tumor of fibroblastic tissue  Metaphyseal region of humerus or tibia mostMetaphyseal region of humerus or tibia most common; for jaw lesions, 90% in molar-ramuscommon; for jaw lesions, 90% in molar-ramus region of mandible.region of mandible.  Most case before age 30; for jaws the mean age isMost case before age 30; for jaws the mean age is 14.14.  Painless swelling with radiolucency of bone.Painless swelling with radiolucency of bone.  Composed of bland collagenous connectiveComposed of bland collagenous connective tissue.tissue.  Sometimes locally destructive and aggressive;Sometimes locally destructive and aggressive; managed surgically according to aggressiveness.managed surgically according to aggressiveness.www.indiandentalacademy.com
  • 52. OsteosarcomaOsteosarcoma 11  Most common primary malignancy ofMost common primary malignancy of bone (non-hematopoietic).bone (non-hematopoietic).  Most are intramedullary; small number areMost are intramedullary; small number are peripheral (juxtacortical), periosteal orperipheral (juxtacortical), periosteal or parosteal.parosteal.  Most often between age 10-20 and locatedMost often between age 10-20 and located in distal femur or proximal tibia;in distal femur or proximal tibia; secondary group > age 50 associated withsecondary group > age 50 associated with Paget’s disease located in axial skeleton.Paget’s disease located in axial skeleton. www.indiandentalacademy.com
  • 53. OsteosarcomaOsteosarcoma 22  Osteosarcoma of jaws accounts for 6-8%Osteosarcoma of jaws accounts for 6-8% of all osteosarcomas; mean age of 33,of all osteosarcomas; mean age of 33, maxilla = mandible.maxilla = mandible.  Swelling and pain, loosening of teeth,Swelling and pain, loosening of teeth, paresthesia and nasal obstruction mostparesthesia and nasal obstruction most common in jaw lesions.common in jaw lesions.  Osteoblastic, chondroblastic andOsteoblastic, chondroblastic and fibroblastic are the most commonfibroblastic are the most common microscopic subtypes.microscopic subtypes. www.indiandentalacademy.com
  • 54. OsteosarcomaOsteosarcoma 33  Radiographic lesions of jaws vary fromRadiographic lesions of jaws vary from lucent to opaque, often ill-defined;lucent to opaque, often ill-defined; spiking or roots, widening of PDLspiking or roots, widening of PDL (common); osteophytic reaction (sun-(common); osteophytic reaction (sun- ray) is seen in 25%.ray) is seen in 25%.  Prognosis linked to the initial attempt atPrognosis linked to the initial attempt at complete removal.complete removal.  Metastasis to regional nodes, lung andMetastasis to regional nodes, lung and brain; > for long bones than jaws.brain; > for long bones than jaws. www.indiandentalacademy.com
  • 55. OsteosarcomaOsteosarcoma 44  For extragnathic sites, pre-op (neoadjuvant)For extragnathic sites, pre-op (neoadjuvant) chemotherapy followed by radical excisionchemotherapy followed by radical excision and post-op (adjuvant) chemotherapy hasand post-op (adjuvant) chemotherapy has increased survival to more than 4 years forincreased survival to more than 4 years for 80%.80%.  Data for jaws sparse estimated 30-50%Data for jaws sparse estimated 30-50% survival. Better outcomes for juxtacorticalsurvival. Better outcomes for juxtacortical tumors compared to intramedullary lesions.tumors compared to intramedullary lesions.  0.2% risk of post-radiation sarcoma for dose0.2% risk of post-radiation sarcoma for dose or 7000 cGy.or 7000 cGy. www.indiandentalacademy.com
  • 56. ChondrosarcomaChondrosarcoma 11  Malignant neoplasm of bone (10%)Malignant neoplasm of bone (10%) characterized by the formation of cartilage.characterized by the formation of cartilage.  Jaw lesions are rare accounting for 1-3% ofJaw lesions are rare accounting for 1-3% of all chondrosarcomas.all chondrosarcomas.  Extragnathic lesions occur predominatelyExtragnathic lesions occur predominately in those > age 50; ileum, femur & humerusin those > age 50; ileum, femur & humerus most common.most common.  Jaw lesions most often in maxilla; reportedJaw lesions most often in maxilla; reported over a wide age range, average age of 41.6over a wide age range, average age of 41.6 and 20% < age 20.and 20% < age 20.www.indiandentalacademy.com
  • 57. ChondrosarcomaChondrosarcoma 22  Most often produces a painless mass; mayMost often produces a painless mass; may be associated with loosening of teeth,be associated with loosening of teeth, epistaxis, nasal obstruction and visualepistaxis, nasal obstruction and visual disturbance.disturbance.  Lesions grades from I-IV; influence onLesions grades from I-IV; influence on outcome; most jaw lesion grade I or II.outcome; most jaw lesion grade I or II.  Size, location and grade influenceSize, location and grade influence outcome; average 5 year survival of 67.5%outcome; average 5 year survival of 67.5% with late recurrence & metastasis reducingwith late recurrence & metastasis reducing long-term survival to less than 44%.long-term survival to less than 44%.www.indiandentalacademy.com
  • 58. Ewing’s SarcomaEwing’s Sarcoma 11  Primary malignancy of bone of uncertainPrimary malignancy of bone of uncertain histogenesis with recent evidence ofhistogenesis with recent evidence of neuroendocrine origin.neuroendocrine origin.  85-90% show reciprocal translocation [t(11;22)85-90% show reciprocal translocation [t(11;22) (q24;q12)].(q24;q12)].  6-8% of primary malignancies of bone; long6-8% of primary malignancies of bone; long bones, pelvis and ribs; jaw or craniofacialbones, pelvis and ribs; jaw or craniofacial lesions are very uncommon (1-2%).lesions are very uncommon (1-2%).  Peak incidence in second decade (50%); 80%Peak incidence in second decade (50%); 80% less than age 20; male slightly > females.less than age 20; male slightly > females. www.indiandentalacademy.com
  • 59. Ewing’s SarcomaEwing’s Sarcoma 22  Rare in blacks.Rare in blacks.  Pain, paresthesia and swelling with looseningPain, paresthesia and swelling with loosening of teeth.of teeth.  Ill-defined lytic lesion; “onion-skin” periostealIll-defined lytic lesion; “onion-skin” periosteal reaction of long bones rare in jaws.reaction of long bones rare in jaws.  Small round cells; PAS positive diastase labile;Small round cells; PAS positive diastase labile; angiotropism and necrosis; MIC2 glycoproteinangiotropism and necrosis; MIC2 glycoprotein detected by immunoperoxidase (CD99).detected by immunoperoxidase (CD99).  Combined surgery, multidrug chemo &Combined surgery, multidrug chemo & radiotherapy 5-year survival 40-80; pelvis worst.radiotherapy 5-year survival 40-80; pelvis worst. www.indiandentalacademy.com
  • 60. Metastatic Tumors to theMetastatic Tumors to the JawsJaws 11  The jaws are regarded as an uncommon siteThe jaws are regarded as an uncommon site for metastasis although a study of autopsiesfor metastasis although a study of autopsies revealed 10/62 (16%) carcinomas involved therevealed 10/62 (16%) carcinomas involved the mandible microscopically though negativemandible microscopically though negative radiographically.radiographically.  80% of reported case are in mandible.80% of reported case are in mandible.  Symptoms include pain, loosening of tooth, aSymptoms include pain, loosening of tooth, a mass or paresthesia (numb-chin syndrome).mass or paresthesia (numb-chin syndrome).  Lucent lesion, well or ill definedLucent lesion, well or ill defined (“moth-eaten”).(“moth-eaten”). www.indiandentalacademy.com
  • 61. Metastatic Tumors to theMetastatic Tumors to the JawsJaws 22  Involvement of alveolus may mimicInvolvement of alveolus may mimic periodontal or periapical disease; occasionalperiodontal or periapical disease; occasional widened periodontal ligament space noted.widened periodontal ligament space noted.  Metastatic carcinomas (especially breast &Metastatic carcinomas (especially breast & prostate) may stimulate new bone formationprostate) may stimulate new bone formation with a resultant radiopaque or mixed lesion.with a resultant radiopaque or mixed lesion.  Most common sites of primary are breast,Most common sites of primary are breast, lung, thyroid, prostate & kidney.lung, thyroid, prostate & kidney.  Stage IV primary (not oral cancer) with aStage IV primary (not oral cancer) with a usually survival of less than one year.usually survival of less than one year. www.indiandentalacademy.com