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