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75 Cards in this Set
- Front
- Back
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What is chondrosarcoma?
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malignant neoplasm of chondroblasts
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How often does chondrosarcoma affect the jaws?
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less than 1%
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Who does chondrosarcoma affect?
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males = females
average age = 33 y/o no site predilection very rare |
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What are the signs of chondrosarcom?
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asymptomatic, usually
more than 1/3 are tender or painful bony-hard mass, slowly enlarging may be lobulated normal overlying mucosa |
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What does chondrosarcoma look like on a radiograph?
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radiolucent or mixed radiolucent/radiopaque
cortical expansion and/or perforation sometimes: sunburst radiopaque, root resorbtion, widened PDL |
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How does chondrosarcoma metastasize?
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via blood vessels, usually to the lungs
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What is treatment for chondrosarcoma?
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radical surgical excision
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What is the prognosis for chondrosarcoma?
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correlated with histologic grade (I-IV)
-Jaw lesions usually Grade I or II -5 year survival rate = 50% |
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How does the prognosis for patients with jaw chondrosarcoma compare to patients with osteosarcoma?
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Worse than osteosarcoma
Extragnathic cases = better than osteosarcoma |
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Chondrosarcoma usually metastasizes to the jaws from where?
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breast, prostate, colon, lung
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What type of cancers are the most common bone tumors in humans?
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metastatic cancers
(NOT primary bone tumors) |
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Who does metastatic cancers that spread to the jaws usually affect?
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males = females
late middle age (45-65 y/o) |
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Where do metastatic cancers of the jaws usually occur?
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posterior mandible, apical regions, tongue
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Jaw cancer compromises what % of oral cancers?
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3%
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What bones are usually affected by metastatic cancers?
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vertebrate, ribs, pelvis, skull
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How do metastatic cancers appear radiographically?
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poorly demarcated radiolucency
moth-eaten radiolucency usually near apex of tooth may show widened PDL often perforates cortex sometimes strong radiodense reaction (especially breast & prostate cancers) |
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How do you see hot spots of metastatic cancers?
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Using technetium scintigraphic bone scan (Tech 99)
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What are the symptoms of metastatic jaw cancer?
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usually painful
may mimic toothache grows out of extraction socket |
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Atypical bone production
Osteogenesis imperfecta abnormality: |
abnormal collagen production
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Atypical bone production
osteopetrosis abnormality: |
lack of osteoclastic activity
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Atypical bone production
bone dysplasia abnormality: |
marrow fibrosis &/or excess bone
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cemento-osseous dysplasis abnormality:
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marrow fibrosis, excess bone, cementum
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reactive osteosclerosis abnormality:
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excess bone in marrow spaces
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reactive periostitis
abnormality: |
excess cortical bone
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What is osteogenesis imperfecta?
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brittle bone disease
mutation in genes for type I collagen AD inheritance, sometimes AR |
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Who does osteogenesis imperfecta affect?
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males = females
infants and young child 1/8,000 births |
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What are effects of osteogenesis imperfecta?
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bowing of legs, arms
angulation of long bones deformity of long bones pathologic fracture hypermobile joints (double-jointed) capillary fragility (pathologic bleeds) osteopenia (low bone density) hearing deficits wormian skull (sutures don't fuse) blue sclera (thin collagen layer) maxillary hypoplasia blue/gray "translucent teeth" |
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What are complications of osteogenesis imperfecta?
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risk of broken bones
anemia (less marrow) tooth fractures C-section for birth Shorten dental crowns Overdenture |
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What is the histopathology for osteogenesis imperfecta?
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immature, irregular trabecular bone
fibrous background diminished amount of marrow teeth have small pulps shell teeth have large pulps |
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What is osteopetrosis aka?
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Albers-Schonberg Syndrome
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What is osteopetrosis?
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inherited failure of osteoclastic function (AD or AR)
number of osteoclasts is normal or high but no bone resorption, gradual thickening of trabeculae/cortex, & gradual sclerosis of bone |
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Who does osteopetrosis affect?
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males = females
infancy (except adult onset type) 1/100,000 persons |
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What are the clinical features of osteopetrosis?
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slow opacification
anemia (spaces fill w/ bone) reduced immune function (fewer hematopoietic cells in small marrow spaces) decreased blood flow to bone (ischemia) - maybe painful pathologic fractures often osteomyelitits, fails to heal (primarily a jaw problem) crimping of nerves in foramina (hearing loss, vision loss, facial palsy) delayed/stopped tooth eruption |
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Infantile osteoporosis
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aka malignant osteopetrosis
severe (usually die in 1st decade) most common form AR inheritance |
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adult osteopetrosis
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benign osteopetrosis
not too severe usually no marrow deficit bone pain is common AD inheritance |
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What is the treatment for osteopetrosis?
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adult = not treated
infant = antibiotics, treat anemias if possible, try to prevent dental infection, hyperbaric therapy |
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cleidocranial dysplasia abnormalities
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missing clavicles, excess teeth
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craniomataphyseal dysplasia abnormalities
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excess mandibular bone, wide long bones
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fibrous dysplasia
abnormalities |
enlargement of affected bone
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cherubism abnormalities
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enlargment of bilateral facial bones
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Paget's disease abnormalities
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alveolar & skull enlargment
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What causes cleidocranial dysplasia?
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defective CBFA1
-chromosome 21 -guides osteoblastic differentation -guides bone formation AD inheritance -40% spontaneous mutations |
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Who does cleidocranial dysplasia affect?
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males = females
childhood and teenage years clavicles, jaws rare |
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Histopathology of cleidocranial dysplasia
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micro: bone looks normal
permanent teeth lack secondary cementum |
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What is the tx for cleidocranial dysplasia?
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surgical exposure and orthodontic reposition of teeth
extraction of teeth with denture construction |
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What is craniometaphyseal dysplasia?
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AD or AR inheritance
bony overgrowth of jaws, skull, sinuses |
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What are the effects of craniometaphyseal dysplasia?
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large head, mandible
flat nasal bridge small sinuses; nasal blockage deafness blindness ocular hypertelorism |
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What do the bones look like in craniometaphyseal dysplasia and what are look-alike disorders?
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altered metaphyses of long bones
erlenmeyer flask shape of ends of long bones (decreased metaphyseal density) sclerosteosis (van buchem disease) craniodiaphyseal dysplasia |
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How does craniometaphyseal dysplasia affect the bones?
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altered metaphyses of long bones
"erlenmeyer flask" shae of ends of long bones decreased metaphyseal density |
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What disorders resemble craniometaphyseal dysplasia?
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-sclerosteosis (van Buchem disease)
-craniodiaphyseal dysplasia |
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What is fibrous dysplasia of bone?
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developmental anomaly of bone
abnormal bone = immature, fibrosis of marrow spaces postzygotic mutation of GNAS 1 gene (guanine nucleotide-binding protein, alpha-stimulating activity polypeptide 1) - explains regional nature of disease |
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Who does fibrous dysplasia affect?
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males = females
7-20 y/o |
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What are the most common sites for fibrous dysplasia?
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posterior maxilla = 85% of H&N cases
jaws |
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What is the histopathology of fibrous dysplasia?
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irregular immature bony trabeculae
fibrous stroma few osteoblasts almost no osteoclasts no capsule becomes more ossified with time |
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What are the clinical/radiographic features of fibrous dysplasia of bone?
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painless bony enlargment, diffuse
- cortical expansion and thinnning ground-glass radiopacity - or irregular opacities - or mixed lucent/opaque poorly demarcated** lamina dura may be hard to see PDL may be thin teeth become separated (remain viable) growth is slow |
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What is the prognosis/treatment for fibrous dysplasia of bone?
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usually "burns out" with adulthood
treat: surgical recountouring, curettage may require multiple procedures |
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What are the clinical variants of fibrous dysplasia?
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*monostatic - one bone involved, 85% of cases, jaws among most common sites
*polyostotic - multiple bones involved, Jaffe-Lichtenstein syndrome, McCune-Albright syndrome |
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Jaffe-Lichtenstein syndrome
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*cafe au lait spots
FD of multiple bones no hormonal problems |
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McCune-Albright syndrome
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clinical variant of polyostotic fibrous dysplasia
relatively uncommon may involve most of skeleton cafe au lait spots sexual precocity hyperthyroidism pituitary adenoma hockey stick deformity of hip |
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Craniofacial fibrous dysplasia
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clinical variant of fibrous dysplasia
adjacent midface bones are only bones involved may cross midline |
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segmental odontomaxillary dysplasia
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clinical variant of fibrous dysplasia
single maxillary quadrant involved radiopaque vertical stripes becomes more ossified with time |
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cherubism is aka
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familial fibrous dysplasia
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What is cherubism?
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developmental anomaly
AD inheritance defective gene on chromosome 16 |
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Who does cherubism affect?
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males = females
1-20 y/o rare |
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Where does cherubism occur?
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posterior jaws
- maybe ribs and humerus |
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What are the clinical features of cherubism?
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usually bilateral; maybe 4 quadrants
greatly expansile radiolucencies - usually multiocular usually stabilizes and slowly regresses face appears greatly enlarged "eyes toward heaven" painless tooth buds moved (sometimes dramatically) wide alveolus |
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What is the histopathology of cherubism?
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loose, immature fibrous stroma
scattered multinucleated giant cells* eosinophils cuffing around small vessels sparse, immature bone like giant cell granuloma old lesions: densely fibrous, fewer giant cells, more mature bone, maybe becomes normal? |
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What is the prognosis/treatment for cherubism?
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begins prior to 5 y/o
enlarges until puberty then "burns out" face is normal by 25-40 y/o can push tooth buds great distances may develop central giant cell granuloma maybe: pathologic fracture treat: none unless pathologic fracture -surgical curretage can be performed -irradiation works but risk of future sarcomas |
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Paget's disease of bone is aka:
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osteitis deformans
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What is Paget's disease?
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Older onset type of "developmental" anomaly
explansile bone enhanced resorption/deposition - possibly from a slow virus, paramyxovirus detected in osteoclasts |
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Who/Where does Paget's disease affect?
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males >>>> females
late middle aged and older maxilla and skull 1/100 persons over 45 y/o but most disease is subclinical |
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What are the clinical features of Paget's disease?
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bowed legs
maybe bone pain-may mimic toothache may get anemia and bleeding - no hematopoietic tissue/platelets, immune deficency deafness and visual loss - pinched nerves in foramina enlarged bone - denture/hat no longer fits elevated alkaline phosphatase - 25% above normal* normal calcium and phosphorus urinary hydroxyproline osteoarthritis |
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What are radiographic features of Paget's disease?
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irregular radiolucency/radiopacity
early stage is radiolucent - osteoporosis circumscripta become more radiopaque over time - cotton wool appearance, cloud-in-the-sky appearance thickened cortex hypercementosis Lincoln's beard (Tech 99) small sinuses |
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What is the histopathology of Paget's disease?
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**chines character bone
-mosaic bone - jigsaw bone immature trabeculae abundant osteoblastic activity lesser osteoclastic activity fibrous background stroma many reversal/cement lines |
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What is the prognosis/treatment for Paget's disease?
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-seldom causes death
-parathyroid hormone antagonists, eg calcitonin, bisphosphonates (to reduce bone turnover) -cytotoxic antibiotics eg plicamycin - inhibit osteoclastic activity (used only in severe cases) -aspirin for pain -antibiotics for osteomyelitis -new dentures or bridgework as maxilla expands -may do base skull surgery to relieve nerves, vessels -Caution! 1-13% risk of osteosarcoma (seldom in jaws) or giant cell tumor (often in jaws) |
