Bone Pathology Flashcards
1
Q
mode of transmission of osteogenesis imperfecta
A
genetic transmission
90% autosomal dominant
2
Q
what genes are implicated in osteogenesis imperfecta?
A
COL1A1 or COL1A2
3
Q
describe the condition of bones in osteogenesis imperfecta
A
defective collagen
abnormal bone mineralization
low tensile strength
very fragile (fractures easily)
4
Q
dental findings of osteogenesis imperfecta
A
- dentinogenesis imperfecta
- bulbous crowns
- constriction at cervical margins of teeth
- absence of pulp chambers
5
Q
ocular findings of osteogenesis imperfecta
A
blue sclera
6
Q
sclera (definition)
A
outermost layer of eyeball
7
Q
choroid (definition)
A
lies deep to sclera
is pigmented (melanin)
8
Q
why are eyes blue in osteogenesis imperfecta?
A
melanin helps to absorb excess light and limits uncontrolled reflection and confusing images (pigment shows through the thin sclera as blue)
9
Q
management of osteogenesis imperfecta
A
- minimize factors that cause fractures
- rehabilitation, orthopedic surgery
- IV bisphosphonates (prevent osteoclastic destruction of bones)
- over dentures
- bone is of poor quality for implants
10
Q
what is the basic defect in osteopetrosis?
A
rare, defect in osteoclast function (bone turnover is lacking)
11
Q
major disease patterns in osteopetrosis
A
infantile (birth, early infancy) = autosomal recessive
adult = milder disease, autosomal dominant
12
Q
changes in bone in osteopetrosis
A
- persistance of bone formation leads to diffuse sclerosis of skeleton
- marrow space filled in by dense bone
13
Q
what results when the marrow space is filled with dense bone in osteopetrosi?
A
- loss of hematopoietic marrow
- pancytopenia (all 3 cell types depleted)
14
Q
pancytopenia (osteopetrosis)
A
- anemia (normocytic) with compensatory hepatosplenomegaly
- infections (due to leukocytopenia)
- bleeding tendencies (due to thrombocytopenia)
15
Q
systemic implications of osteopetrosis
A
- increase in bone density with absence of medullary bone
- increase width of cranium
- proptosis due to deposition in orbital cavity
- blindness (optic canal compressed)
- deafness (compression fo CN 8)
- facial palsy (compression of stylomastoid foramen)
- hypertrophy of bones (relative microsomia)
16
Q
blindness in osteopetrosis is due to…
A
optic nerve compression
17
Q
deafness in osteopetrosis is due to…
A
CN 8 compression
18
Q
facial palsy in osteopetrosis is due to…
A
compression of stylomastoid foramen
19
Q
oral complications of osteopetrosis
A
- impacted teeth in recessive form of disease
- poor oral health, dental decay, infections following dental extractions
- mandibular osteomyelitis with multiple draining sinus tracts
20
Q
management of osteopetrosis
A
1.bone marrow transplant
2. support measures to minimize infection / fracture risks
3. aggressive treatment of osteomyelitis (antibiotics, hyperbaric O2)
4. interferon gamma-1b with calcitriol and restricted calcium intake
21
Q
interferon gamma-1b in the treatment of osteopetrosis has shown to…
A
reduce bone mass
decrease prevalence of infections
lower nerve compression frequency
22
Q
what gene mutation results in cleidocranial displasia?
A
RUNX2 gene
23
Q
transmission of cleidocranial dysplasia
A
uncommon autosomal dominant condition
40% spontaneous mutations
24
Q
clinical findings of cleidocranial dysplasia
A
- affects skull, jaws, CLAVICLES
- large skulls with prominent forehead (frontal and parietal bossing)
- hypertelorism
- depressed bridge and wide base of nose
- high narrow or cleft palate
- hypopalstic maxilla
- primary dentition retained as permanent teeth do not erupt
25
radiographic findings of cleidocranial dysplasia
can see retained primary teeth with unerupted perm teeth or super teeth
edentulous patient with impacted teeth
26
management of cleidocranial dysplasia
1. surgical and ortho care to correct skeeltal relations
2. remove supernuemrary teeth and bring permanent teeth into proper relatoin
27
prognosis of cleidocranial dysplasia
good
normal life span
28
clinical features of osteoporotic bone marrow defect
1. middle aged to older females
2. incidental finding - no swelling or pain
29
radiographic features of osteoporotic bone marrow defect
asymptomatic ill-defined radiolucency in BODY OF MANDiBLE AT OLD EXTRACTION SITE
30
wht tissue is encountered if osteoporotic bone marrow is biopsied?
2. may resemble metastatic disease (biopsy sometimes necessary)
31
when is a biopsy for osteoporotic bone marrow necessary?
when the patient gives history (current or past) of cancer
need to rule out
32
what is seen microscopically for osteoporotic bone marrow?
fatty and hematopoietic marrow
33
treatment of osteoporotic bone marrow defect?
pack with bone chips and bring back bone quality - follow up after
34
radiographic findings of idiopathic osteosclerosis
1. ENOSTOSIS / DENSE BONE ISLAND
2. asymptomatic lesion discovered on routine radiographs (PDL space continuous)
3. RADIOPAQUE, NO EXPANSION
4. PREMOLAR - MOLAR REGION MOST COMMON
5. margins may be sharp or blended with adjacent bone
35
treatment of idiopathic osteosclerosis?
don't need to biopsy
will show dense vital bone
36
what are the clinical features of condensing osteitis
1. localized bone sclerosis associated with apices of teeth with pulpitis (large carious lesion or deep coronal restoration)
2. associated with area of inflammation is critical or diagnosis (pulpitis and osteitis: inflammation)
3. will have pain
37
what are the radiographic features of condensing osteitis
1. seen in tooth with pulpitis
2. bone responts with calcification (fights back by forming more bone) - follows contour of the tooth with sclerosis
38
how are the bone changes with condensing osteitis affected by either extraction or root canal treatment of the tooth?
1. can reverse completely or partially
2. removed the source of the focus of infection, normal routine bone remodeling will make that bone go away
3. 85% of cases regress totally or partially upon removal of focus of infection
39
describe the clinical features of paget's disease of bone
1. osteitis deformans
2. abnormal resorption and deposition of bone
3. distortion and weakening of bone
4. unknown etiology
5. older patients, male predilection
6. many asymptomatic, but bone pain may be present
7. most cases polyostotic
8. affected bone thickened but weak
9. involvement of femurs, simian stance develops due to BOWING OF LEGS
40
radiographic features of paget's disease
cotton wool appearance of skull with areas of osteoporosis circumscripta
41
histopathological features of paget's disease
irregular trabeculae with resetting and reversal lines - "mosaic" pattern
dark lines are reversal lines
42
jawbone / teeth findings of paget's disease
10-15% of affected patients
maxilla ? mandible
HYPERCEMENTOSIS OF TEETH
43
what serum marker is elevated in paget's disease of bone?
markedly elevated serum alkaline phosphatase
44
treatment of paget's disease
no good therapy
bisphosphonates reduce bone turnover with normalization of serum alkaline phosphatase levels, decrease bone pain and improve quality of life
45
complications of paget's disease
1. chronic and progressive
2. patients to be monitored for development of giant cell tumor of bone as well as malignant bone tumors, especially osteosarcoma
3. hypercementosis and complicates tooth extraction
4. unfavorable results with dental implants
46
demographics affected by central giant cell granuloma
2nd-4th decade of life
2:1 female predilection
47
clinical features of central giant cell granuloma
1. benign lesion of jaws
2. unilocular when small; larger lesions often multilocular and expansile
3. central - intraosseous process (inside jaw)
4. try pulp testing first, ask any trauma, teeth would test vital
48
radiographic features of central giant cell granuloma
1. mandible, often crossing the midline
2. thin wispy trabeculae perpendicular to outer cortex
3. multilocular radiolucency of the mandible, crossing the midline (buccal expansion)
49
histopathologicla features of central giant cell granuloma
vascular CT with copious amounts of erythrocytes and multinucleated giant cells
50
management of central giant cell granuloma
aggressive currettage
intralesional steroid injections
calcitonin
51
possible complication in older patients with central giant cell granuloma
possiblity of brown tumor of hyperparathyroidism that should be explored
52
what is a brown tumor of hyperparathyroidism?
1. take out all the calcium from the bone and put into the blood
2. osteitis fibrosa cystica
3. histology identical to the central giant cell granuloma with many erythrocytes
4. hemoglobin converts into hemosiderin pigement (looks brown in color due to pigment)
53
jawbone findings in brown tumor of hyperparathyroidism
1. ground glass appearance, loss of lamina dura, brown tumor
2. trabeculae thin out (bone looks homogenous, opaque ground glass)
54
demographics affected by cherubism
childhood
autosomal dominant condition?
55
clinical findings of cherubism
1. painless, bilateral expansion of jaws, especially in the mandible
2. "chubby" due to bone expansion, upward turn of eyes
56
radiographic features of cherubism
1. bilateral multilocular radiolucencies of posterior mandible
2. less frequently, maxillary involvement
3. some unilateral cases described
4. often significant anterior placement of teeth
57
management of cherubism
1. lesions regress after puberty
2. by 4th decade, facial features almost normal
3. residual deformity may be surgically removed
58
etiology of traumatic (simple) bone cyst
1. hemorrhagic bone cavity
2. idiopathic condition seen in 1st and 2nd decase
3. questionable relation to trauma
59
demographics affected by traumatic bone cyst
male predilection; posteiror mandible
60
radiogrpahic features of traumatic (simple) bone cyst
well circumscribed radiolucency with scalloping between roots
lamina dura still intact, lack of expansion, intact cortex
61
intraoperative findings of traumatic bone cyst
1. empty cavity is found within bone (often inferior dental canal may be noted at the bottom of the cavity)
2. difficult to obtain lesional tissue (scant amount of serous fluid)
62
management of traumatic bone cyst
1. empirically, the recommendation has been to enter the lesion, establish the diagnosis, then induce bleeding
2. supposedly the hemorrhage organizes and the lesion heals
63
fibrous dysplasia (definition)
developmental, tumor like lesion
hard bony growth
64
etiology of fibrous dysplasia
post zygomatic mutation of tumor suppressor (GNAS) gene
65
can the post-zygotic mutation of fibrous dysplasia be passed onto children?
no
66
types of fibrous dysplasia
monostatic form
polyostotic fibrous dysplasia
67
monostatic form of fibrous dysplasia
most cases are late post zygotic and result in this form
68
polyostotic fibrous dysplasia
early post-zygotic mutation causes this form
69
demographics affected by fibrous dysplasia
first or second decade
no sex predilection
70
are most cases of fibrous dysplasia monostatic or polostotic?
monostatic
71
jawbone manifestations of fibrous dysplasia
jaws are among the most commonly affected bones
72
maxilla or mandible more affected by fibrous dysplasia?
maxill
73
cranial bone manifestations of fibrous dysplasia
1. maxillary lesions may involve adjacent facial bones, including sphenoid, zygoma, occipital
2. known as craniofacial fibrous dysplasia
3. more severe presentation
4. marked facial deformity
74
radiographic appearance of fibrous dysplasia
ground glass
poorly defined, blended margins
lamina dura lost over time
75
loss of lamina dura in hyperparathyroidism vs fibrous dysplasia
early generalized loss of lamina dura is characteristic of hyperparathyroidism
lamina dura is lost over time in fibrous dysplasia
76
histopathology of fibrous dysplasia
irregularly shaped trabeculae of immature bone ("chinese letter")
trabeculae somewhat evenly spaced
77
polyostotic fibrous dysplasia
many bones involved
78
jaffe type of polyostotic fibrous dysplasia
two or more bones affected
cafe-au-lait spots
79
mccune type of polyostotic fibrous dysplaisa
2+ bones affected
cafe-au-lait spots
exocrine disturbances manifest as precocious puberty
intramuscular myxomas (mazabraud syndrome)
80
treatment of fibrous dysplasia
1. small lesions may not need treatment or may be recovered by en bloc resection
2. significant cosmetic or functional deformity may require an attempt at surgical reduction (debulking)
3. sometimes the disease stabilizes iwth skeletal maturation
81
potential complications of fibrous dysplasia
1. 25-50% of surgically treated lesions show regrowth, particularly in younger patients
2. malignant transofrmation is rare and reported in lesions that have received radiaiton therapy
82
most important thing to remember in treatment of fibrous dysplasia
never radiate anything that is a benign lesion
83
what is the most important thing to remember about cemento-osseous dysplasia
never perform endodontic treatment on these patients!
84
ethnic predilection of cemento-osseous dysplasia
african american females
southeast asia
85
variants of cemento-osseous dysplasia
periapical (most common, mild)
focal (moderate)
florid (severe)
86
radiogrpahic features of cemento-osseous dysplasia
1. usually detected on routine radiographs
2. mandibualr anteiror region
3. initially, radiolucencies at apicies of several teeth, with gradual central opacity developing
4. radiopacity is completely in bone (not part of root)
87
nature of cemento-osseous dysplasia disease
osteolytic --> osteoblastic --> maturative
88
treatment of cemento-osseous dysplasia
none necessary! do nothing at all!
89
focal cementoosseous dysplasia is usually detected on
routine radiographcs
90
focal cemento-osseous dysplasia - location
body of mandible
91
difference between focal cemento-osseous dysplasia and odontoma / central ossifying fibroma
when treating odontoma or central ossifying fibroma, has thick capsule surrouding. easy to separate from surroudnign bone
nto case for florid type cemento-osseous dysplasia (not well defined)
92
treatment for focal cemento-osseous dysplasia
no treatment required
93
complications associated with florid type fo cemento-osseous dysplasia
1. more severe expression fo the cemento-osseous dysplasias
2. affects multiple quadrants of jaws
3. radiolucencies with multiple cotton wool radiopacities in at least 2 quadrants of the jaws
4. lesions become mroe radiodense with time
94
central ossifying fibroma
uncommon benign neoplasm that probably arises from periodontal ligament fibrobalsts
95
demographics of central ossifying fibroma
mandibular premolar / molar region
female
3rd to 4th decade
96
clinical features of central ossifying fibroma
actual tumor!
swelling may be present if lesion is large
97
radiographic features of central ossifying fibroma
1. well circumscribed radiolucency with vairable amount of central opacity
2. range from almost purely radiolucent or very radiodense with a defined lucent border
98
management of central ossifying fibroma
treatment consists of enucleation - lesions tend to shell out as one mass
99
what diseases may the central ossifying firboma like tumor be associated with?
hyperparathyroidism
gnathodiaphyseal dysplasia
familial gigantiform cementoma
100
demographics of trabecular variant of juvenile ossifying fibroma
11years
101
demogrpahics of psammamatoid variant of juvenile ossifying fibroma
22 years
102
common site of juvenile ossifying fibromas
predominantly maxillary tumors
70% of psammamatoid variant arises in bones of the orbit, frontal, and paranasal sinuses
103
clinical features of juvenile ossifying fibroma
1. progressive enlargement
2. symptoms associated with impingement on adjacent structures
3. most aggressive tumors in infants and young hcildren
104
management of juvenile ossifying fibroma
1. small lesions = local excision with thorough currettage
2. large lesions = wide resection
105
histopathology of two variants of juvenile ossifying fibroma
trabecular = fine trabeculae
psammamatoid = small sand grains, calcificaitons
106
osteoma
benign osseous tumor, usually affecting membranous bone
107
location of osteoma
parnaasal sinus involvement is common
jaw lesions are usually associated with condylar area
108
clinical features of osteoma
painless, slow enlarging
109
relationship of osteoma with gardner sydnrome
autosomal trait
one of the must intestinal polyposis syndrome
characterized by osteomas of the facial bone
epidrmoid cysts and desmoid tumors
110
inheritance of gardner sydnrome
autosomal dominant trait (uncommon)
111
clinical features of gardner sydnrome
1. one of the multiple intestinal polyposis sydnromes
2. characterized by osteoms of the facial bone
3. epidermoid cysts and desmoid tumors
112
radiographic features of gardner syndrome
2. radiographically, the osteoma of gardner sydnrome could resemble florid cemento-osseous dysplasia or osteitis deformans
2. may see impacted supernumerary teeth or less commonly odontomas
113
complications of gardner sydnrome
1. the most significant aspect of the sydnrome is the development of precancerous polyps of the colon
2. 50% of patients develop adenocarcinoma of the colon by 30 years of age
3. also develop multiple epidermoid cysts on the scalp
114
prophylaxis of gardner syndrome
1. prophylactic colectomy
2. removal of costmetically problematic cysts and osteomas
3. genetic counseling
115
incidence of osteosarcoma
most common primary bone malignancy - twice as common as chondrosarcoma
only 900 new cases int he US annually
116
age predilection in jaw bones vs long bones
osteosarcoma of long bone = mean 18 years
osteosarcoma of jaws = mean 28 years
117
clinical features of osteosarcoma
pain
swelling
loss of teeth
paresthesia
118
radiogrpahic features of osteosarcoma
1. mixed radiopaque / radiolucent lesions with ill defined borders
2. widened PDL of teeth
3. spiking root resorption
4. sun brts pattern of periosteal expansion
119
management of osteosarcoma
radical surgery
preceded by neoadjuvant chemoterapy in high grade tumors
120
two other examples of primary jawbone malignancy
chondrosarcoma
ewing sarcoma
121
chondrosarcoma
adult males
10% in jaw bones
122
ewing sarcoma
children and adolescents
commonest primary bone malignancy in kids
123
metastatic tumros are often called
seocndaries
124
incidence rates of metatistic tumors
mandible = 61%
maxilla = 24%
soft tissue = 15%
125
role of baston's plexus in spread of tumor
1. odler and elderly
2. mobility of spread, paresthesia (NUMB CHIN)
3. irregular widening of PDl, moth eaten apperance (could easily be misdiagnosed as periodontal disease)
126
majority of metastatic tumors in jaw come from
breast
lunk
kidney
colon
prostate
