Chapter 14: Bone Pathology part I Flashcards
what is necessary for diagnosis of focal osteoporotic marrow defects? what is the treatment?
- incisional biopsy
- no treatment necessary
how is idiopathic osteosclerosis diagnosed?
- can be made based on history, clinical features, and radiographic findings
- biopsy is considered only if there are symptoms, contingued growth, or cortical expansion
idiopathic osteosclerosis affects approximately ___% of americans
5%
___ is a group of heritable disorders characterized as a defect in type I collagen maturation
osteogenesis imperfecta
what is the treatment of bone anomalies in cleidocranial dysplasia?
- no treatment
- patients usually function well regardless
- patients are usually unaware they have a syndrome
are focal osteoporotic marrow defects symptomatic? how are they usually discovered?
no, and they are typically found incidentally on radiographs
90% of idiopathic osteosclerosis cases are in what location? is there cortical expansion?
- mandible
- no cortical expansion
infantile osteopetrosis is a ___ disease
severe
associated with a poor prognosis
central giant cell granuloma is considered a ___ lesion, but some cases demonstrate aggressive behavior
nonneoplastic
what is the most common clinical manifestation of aneurysmal bone cysts?
swelling that has developed rapidly
pain is often reported
cherubism causes marked widening and distortion of ___, which may lead to ___
- alveolar ridges
- failure of tooth eruption amongst other things
osteogenesis imperfecta as characterized by bowed long bones
with jaw involvement of paget’s disease, is it more common in the maxilla or mandible? what is the presentation?
- maxillary disease > mandibular disease
- enlargement of middle 1/3 of the face; severe cases results in leontiasis ossea, or lionlike facial deformity
- alveolar ridges are symmetrical and grossly enlarged
- patients classically complain dentures no longer fit (or, if present, increased space between teeth)
paget’s disease affecting the skull leads to a progressive increase in ___
the circumference of the head
describe benign bone neoplasia
- asymptomatic
- symmetrical
- does not metastasize
- grows slowly and by expansion; displaces teeth and expands the cortex
patients with osteopetrosis discovered at what time in life will have malignant osteopetrosis?
at birth or early infancy
what are the radiographic features of central giant cell granuloma? are radiographs diagnostic?
- radiolucent lesions which may be multilocular or unilocular
- typically well-delineated
- noncorticated margins
- not diagnostic
cherubism
what is the prognosis of central giant cell granuloma? is there a risk for metastasis?
- long term prognosis is good
- no risk for metastasis
describe malignant bone neoplasia:
is it symptomatic or asymptomatic? grows slowly or quickly? symmetrical or asymmetrical? capable of metastasis?
- usually symptomatic
- grows more rapidly than benign
- often asymmetrical
- capable or metastasis
how are most central giant cell granulomas discovered?
- most are asymptomatic and discovered during routine xray or due to painless bone expansion
what is the prognosis of aneurysmal bone cysts?
good
osteopetrosis
development of osteosarcoma occurs in up to ___% of paget’s disease patients. most develop in what bones? what is the prognosis if an osteosarcoma develops?
- 10%
- most develop in the pelvis or long bones of lower extremities
- very aggressive and associated with a poor prognosis
radiographically, how does paget’s disease present?
- patchy sclerotic areas = cotton wool appearance
- teeth often have hypercementosis
osteopetrosis is also called ___
marble bone disease
most aneurysmal bone cyst jaw lesions are found in patients around what age and what location? male or female predilection?
- 20 years
- posterior mandible
- M = F
jaw involvement of paget’s disease occurs in ___% of cases
20%
___ is an area of hematopoietic marrow that produces a radiolucency
focal osteoporotic marrow defect
patients with paget’s disease will have high elevations in ___ and normal levels of ___ and ___
- serum alkaline phosphatase
- normal calcium and phosphorus
what are the radiographic features of osteogenesis imperfecta?
- osteopenia (low bone density)
- bowing
- angulation or deformity of long bones
- multiple fractures
- wormian bones in the skull
microscopic findings of cherubism are identical to those found in ___
central giant cell granuloma
osteopetrosis results from a defect in remodeling caused by ___
a failure of normal osteoclast function
cherubism occurs between what ages? how does it progress?
- 2-5 years old
- clinical alterations progress until puberty, then stabilize and slowly regress
is an aneurysmal bone cyst a true cyst?
no; there is no epithelial lining
what is the treatment of paget’s disease? what cases is treatment unnecessary?
- not given in asymptomatic patients with limited involvement
- NSAIDs are given for pain
- pharmacologic antiresorptive agents are given for symptomatic patients or those with alkaline phosphatase levels 50% above normal
- bisphosphonates often used
___ is a common complaint of paget’s disease
- bone pain
- pagetic bone forms near joints and promotes osteoarthritic changes, with associated joint pain and limited mobility
aneurysmal bone cysts are typically seen in ___ bones; it is uncommon in the ___
- long
- jaw
what do mandibular lesions look like in cherubism? what about maxillary?
- painless, bilateral, posterior, and expansile
- maxillary involvement occurs posteriorly as well
- in severe cases, entire maxilla and mandible are involved
which of the 4 major types of osteogenesis imperfecta is characterized as not compatible with life
type II
osteogenesis imperfecta as characterized by wormian bones of the skull (sutures)
what are the radiographic findings of traumatic bone cysts?
- well-delineated
- radiolucent
- margins can be ill- or well-defined
- when several teeth are involved, the defect shows domelike projections that scallop between the roots; this feature is highly suggestive
- radiographic findings are suggestive but not diagnostic
what type of treatment is contraindicated in aneurysmal bone cyst cases?
irradiation
patients with cleidocranial dysplasia will have prolonged retention of ___, and delayed or failur of eruption of ___
- deciduous teeth
- permanent teeth
development of ___ is a recognized complication of paget’s disease
osteosarcoma (malignant bone tumor)
what 6 things does infantile osteopetrosis result in?
- marrow failure
- frequent fractures
- cranial nerve compression
- facial deformities
- delayed tooth eruption
- osteomyelitis post tooth infraction
describe the clavicle characteristics in cleidocranial dysplasia
- clavicles are usually present and show varying degrees of hyploplasia
- clavicles are absent in 10% of cases
- unusual mobility of shoulders
___% of adult osteopetrosis are asymptomatic
40%
___ is a skeletal disorder with a marked increase in bone density
osteopetrosis
cherubism is a developmental jaw condition with autosomal ___ inheritance
dominance
the histopathology of central giant cell granuloma is identical to ___ and ___. what is the histology?
- a brown tumor of hyperparathyroidism (so patients MUST be evaluated for this condition) and lesions of cherubism
- histology is a cellular, vascular stroma with prominent multinucleated giant cells
describe the prognosis of a patient with cherubism
unpredictable
- usually, the lesions show varying degress of remission and involution after puberty
- by age 30, most patient’s facial features are normal
- however, some patients are left with facial deformities
- early surgical intervention with curettage has lead to both good results or rapid regrowth with worsening deformity; therefore, therapy hasn’t been determined
what 3 things can idiopathic osteosclerosis be confused with, and what are the distinguishing features of each?
- condensing osteitis - associated with an infection
- focal cemento-osseous dysplasia - has radiolucent rim
- cementoblastoma - fused with the tooth
radiation therapy is contraindicated for patients with cherubism due to risk of ___
postirradiation sarcoma
in osteogenesis imperfecta cases, upon fractures, what is the healing process?
healing occurs, but it may heal inappropriately
what are the radiographic features of cherubism?
- multilocular
- expansile
- radiolucent
- the appearance is virtually diagnostic due to the bilateral location in a young person
what is the treatment for osteopetrosis?
- bone marrow transplant is the only hope
- only 50% of those eligible survive the transplant
what are the radiographic features of infantile osteopetrosis?
- widespread increase in skeletal density (increased radiopaque)
- roots of teeth are difficult to visualize because of the density of the surrounding bone
a minority of central giant cell granuloma cases are aggressive and associated with what 3 things?
pain, parasthesia, and perforation of the cortical plate
osteopetrosis
what are the two major clinical patterns of osteopetrosis?
infantile and adult
___ is the most common type of inherited bone disease
osteogenesis imperfecta
what is the etiology of aneurysmal bone cysts?
unknown
how are traumatic bone cysts treated? what is the prognosis?
- surgical exploration is curative; there will be rapid obliteratoin of the defect by new bone formation
- periodic xrays should be taken until complete resolution has occurred; the prognosis is excellent
why is a traumatic bone “cyst” a misnomer?
it is not a true cyst because the lesion does not have an epithelial lining
malignant bone neoplasia invade and destroy ___
adjacent structures (cortex)
ragged/poorly defined margins
focal osteoporotic marrow defect
adult osteopetrosis is also called ___
benign osteopetrosis
numerous unerupted permanent and supernumerary teeth are common in what condition?
cleidocranial dysplasia
how are traumatic bone cysts diagnosed?
- surgical exploration is necessary
- little or no tissue will be obtained, so the diagnosis is made with the clinical and radiographic features together with surgical findings
- during surgery, the wall of the cavity should be curetted to rule out thin-walled lesions
which of the 4 major types of osteogenesis imperfecta is characterized as the most common, mildest, blue sclera throughout life
type I
what is the goal of therapy for osteogenesis imperfecta?
symptomatic treatments and management of fractures
most conditions of idiopathic osteosclerosis arise in ___ years and remain ___
teenage, static
how are aneurysmal bone cysts treated?
- curettage or enucleation
- surgical defect typically heals within 1 year without the need of bone grafting
- recurrence ranges from 10-60% depending on the study and is usually due to inadequate removal of the first lesion
what are the distinctive, occasional findings noted in the oral cavity of patients with osteogenesis imperfecta?
- teeth with a blue to brown translucence
- premature pulpal obliteration
- class III malocclusion
is there jaw expansion associated with focal osteoporotic marrow defects?
no
___ is a relatively common disease characterized by abnormal resorption and deposition of bone
paget’s disease of bone
central giant cell granulomas are more common in what location of the jaw?
anterior jaw (more common in mandible) and frequently crosses the midline
how do focal osteoporotic marrow defects present radiographically?
- radiolucent lesion that varies in size
- typically has ill-defined borders with fine central trabeculations
likely filled with blood
no epithelial lining
aneurysmal bone cyst
what is the exposed sclera caused by in cherubism?
- involvement of the infraorbital rim and orbital floor = eyeballs tilted upward
- stretching of the upper facial skin = pulls lower lid downward
___ is a focal area of increased radiodensity that is of unknown cause and cannot be attributed to anything else
idiopathic osteosclerosis
what are significant complications of adult osteopetrosis?
fracture and osteomyelitis after tooth extraction
osteogenesis imperfecta as characterized by blue sclera
which of the 4 major types of osteogenesis imperfecta is characterized as the most severe form, 1/3 survive into adulthood
type III
what is the treatment of idiopathic osteosclerosis if the lesion is discovered during adolescence?
- periodic xrays are prudent until the area stabilizes
- after that, no treatment is necessary
the radiographic features of focal osteoporotic marrow defect are similar to ___, but it is actually not a ___
- intraosseious neoplasm
- it is not a pathology
in the jaws, traumatic bone cysts are found in pateints of what age? which jaw? are they symptomatic?
- 10-20 years old
- almost exclusively in the mandible
- asymptomatic; 20% have swelling
central giant cell granuloma
what is the appearance of someone with cleidocranial dysplasia?
- short stature; big head
- pronounced frontal bossing
- ocular hypertelorism
- broad base of nose
likely an empty cavity
no epithelial lining
traumatic bone cyst
most cases of central giant cell granuloma occur before what age? male or female? mandible or maxilla?
- before age 30
- F>M
- mandible > maxilla
what are the signs/symptoms of osteogenesis imperfecta?
- bone fragility
- blue sclera
- altered teeth
- hearing loss (hypoacusis)
- long bone and spine deformities
- joint hyperextensibility
75% of focal osteoporotic marrow defects occur in males or females? typically in what location?
- females
- typically in posterior mandible
what is common during surgery of an aneurysmal bone cyst?
venous blood wells up and there may be bleeding
appearance is like a blood-soaked spongue
___ is a syndrome complex characterized by dental and clavicle abnormalities (hyploplastic clavicles)
cleidocranial dysplasia
the majority of traumatic bone cysts are found where?
in long bones
most patients with infantile osteopetrosis die during the first ___ of life
decade
paget’s disease of bone
cleidocranial dysplasia occurs in 1 out of every ___ people
1,000,000
cleidocranial dysplasia
how does adult osteopetrosis compare to the infantile form?
- is it discovered later in life and has less severe manifestation
- disease is mild and has long-term survival
- marrow failure is rare
traumatic bone cyst is also called a ___
simple bone cyst
which of the 4 major types of osteogenesis imperfecta is characterized as resembling type I, slightly more severe (more fractures)
type IV
what is the most widely accepted etiology of traumatic bone cyst?
- trauma-hemorrhage theory
- trauma to the bone which is insufficient to cause a fracture, resulting in intraosseous hematoma
- if the hematoma does not undergo organization and repair, it may liquefy and result in a defect
what is the treatment of central giant cell granuloma? what is the recurrence?
- curettage with a recurrence of 20%
- recurrence likelihood is greater in young patients
- most recurrent lesions resond to further curettage
- new treatments are used with aggressive tumors, such as injections of corticosteroids
paget’s disease of bone
what are the characteristic facial features of charubism?
- bilateral involvement of the posterior mandible that produces chubby cheeks
- wide rim of exposed sclera below the iris causes an “eyes upturned to heaven” appearance
what are characteristics of bone in osteogenesis imperfecta?
- thin cortex
- fine trabeculation
- diffuse osteoporosis
patients with cleidocranial dysplasia have ___ shaped teeth
abnormally
central giant cell granuloma
what are the most commonly affected bones in paget’s disease of bone?
- vertebrae
- pelvis
- skull
- femur
___ is an intraosseous accumulation of blood-filled spaces surrounded by connective tissue
aneurysmal bone cyst
osteogenesis imperfecta as characterized by brown translucence of teeth and radiographs showing shell teeth with thin dentin and enamel of normal thickness
how does idiopathic osteosclerosis present radiographically?
- well-defined, round or elliptical, radiopaque
- usually associated with root apex, 20% aren’t
- vary from 3mm to 2cm in greatest diameter
- there is NO radiolucent rim surrounding the lesion
in paget’s disease, involvement of weight-bearing bones leads to ___, resulting in a ___ stance
- bowing
- simian (monkeylike)
osteogenesis imperfecta affects 1 in ___
8000
paget’s disease is polyostotic. what does that mean?
it affects more than one bone
what medications might patients with osteogenesis imperfecta be on?
bisphosphonates
reserved for moderately to severely affected patients
what is the cause of paget’s disease?
unknown
paget’s disease of bone result in what?
distortion and weakening of affected bones
what population does paget’s disease often affect?
M>F
caucasians > african americans
1/125 people > 45
___ is a benign, empty or fluid filled cavity within bone
traumatic bone cyst
are the teeth affected by traumatic bone cysts vital?
yes
___ is slowly and chronically progressive, but rarely causes death
paget’s disease
idiopathic osteosclerosis
what is the treatment of dental problems associated with cleidocranial dysplasia?
- can be difficult
- can include full-mouth extractions with dentures or various other prosthodontics, ortho, and oral surgery related options
what are the palate characterics of someone with cleidocranial dysplasia?
- high-arched palate
- increased prevalence of cleft-palate
pt also has hypoplastic clavicles
cleidocranial dysplasia
what are the radiographic features of aneurysmal bone cysts?
- radiolucent lesion with marked cortical expansion and thinning
- usually unilocular; can be multilocular
- borders are variable; can be well-defined or diffuse
- frequently described is a “blow-out” or ballooning distention of the countour of the affected bone
there is no association between focal osteoporotic marrow defect and any ___ disorder
hematologic
