Bone Disorders Flashcards

1
Q

most common primary bone malignancy

A

osteosarcoma

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2
Q

most common general location of osteosarcoma

A

long bones

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3
Q

most common dental relevant location of osteosarcoma

A

mandible

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4
Q

most common age for osteosarcoma

A

10-25 (jaw lesions a decade later)

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5
Q

osteosarcoma symptoms

A
  • pain, paresthesia in mandible
  • nasal obstruction, epistaxis (nose bleeds) in maxilla
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6
Q

radiographic features - osteosarcoma

A
  • sunburst radiopaque lesions
  • PDL widening
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7
Q

osteosarcoma histology

A
  • sarcomatous stroma
  • production of osteoid and bone
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8
Q

osteosarcoma - differential diagnosis

A
  • chondrosarcoma
  • osteoblastoma
  • ossifying fibroma
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9
Q

how can you tell the difference between osteosarcoma and chondrosarcoma?

A
  • histologically chondrosarcoma has cartlaginous structures and does NOT have osteoid and bone in the stroma
  • chondrosarcoma does not show distinctive deomographics like osteosarcoma
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10
Q

osteosarcoma - treatment

A

radiation & chemotherapy

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11
Q

chondrosarcoma

A

malignant neoplasm devoid of osteoid and bone with cartlagenous structures

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12
Q

is chondrosarcoma common or rare?

A

rare

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13
Q

chondrosarcoma clinical features

A
  • expansion
  • loose teeth
  • nasal discharge
  • pain may be present
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14
Q

chondrosarcoma histological features

A
  • fully developed cartilage
  • NO osteoid or bone
  • very large nuclei
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15
Q

chondrosarcoma radiographic features

A
  • “cotton wool” calcifications
  • widened PDL may be present
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16
Q

mesenchymal chondrosarcoma

A

dental relevant subtype of chondrosarcoma - more commonly occurs in the mandible

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17
Q

fibrosarcoma

A

malignant neoplasm of fibroblastic cells

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18
Q

fibrosarcoma: common or rare

A

rare

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19
Q

fibrosarcoma - age

A

over 40

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20
Q

what is an important clinical treatment that could potentially cause fibrosarcoma?

A

radiation therapy

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21
Q

clinical features of fibrosarcoma

A
  • same as any malignancy - NOT characteristic
  • mass or swelling common
  • pain, paresthesia
  • trismus
  • MIMICS: SCC which is much more common
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22
Q

can fibrosarcoma occur in children

A

yes (childhood head and neck fibrosarcoma is a distinct entity)

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23
Q

fibrosarcoma - histological features

A
  • interlacing connective tissue cells (looks like interlaced cable wires)
  • cells arranged in fascicles often with a “heringbone” pattern
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24
Q

what are the two forms of fibrosarcoma in children

A
  • desmoplastic
  • medullary
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25
Q

which childhood form of fibrosarcoma behaves like the adult form?

A

desmoplastic fibrosarcoma

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26
Q

which childhood form of fibrosarcoma is less aggressive

A

medullary fibrosarcoma

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27
Q

how do you treat fibrosarcoma?

A

complete excision (get it out, radical resection advised)

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28
Q

fibrosarcoma prognosis

A

not good

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29
Q

langerhans cell histocytosis (LCH)

A

neoplastic proliferation of langerhans cells with a wide spectrum of biologic behavior

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30
Q

what are the three categories of LCH?

A
  1. chronic focal/”eosinophilic granuloma”/unifocal
  2. chronic disseminated/”Hand-Schüller-Christian disease”/multifocal unisystemic
  3. acute disseminated/”Letterer-Siwe disease”/multifocal multiple system

need to know the different names

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31
Q

characteristics of the chronic focal/ “eosinophilic granuloma”/unifocal type of LCH

A
  • solitary granuloma
  • no soft tissue or organ involvement
  • easily treatable
  • M > F
  • children & young adults
  • radiographically - teeth appear to “float in air”, unilocular lesions in skull appear “punched out”
  • simple excision or curettage
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32
Q

characteristics of chronic disseminated/ “Hand-Schüller-Christian disease”/multifocal unisystemic type of LCH

A
  • multifocal (affects multiple bones & organs)
  • multiple lesions
  • multiple sites
  • diabetes insipidus
  • hisotlogiccally - histocytes & eosinophils
  • surgery, radiation, chemotherapy, steroids
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33
Q

characteristics of acute disseminated/”Letterer-Siwe syndrome”/mulitifocal multiple system type of LCH

A
  • infants
  • lethal form
  • multiple organ involvement
  • histologically - “foamy” cytoplasm
  • chemotherapy
34
Q

immunohistochemistry - LCH will stain positive for?

A

CD1

35
Q

what is the name of the staging system for LCH

A

Kemp staging system

36
Q

central giant cell granuloma of jaw

A

distinct diagnostic entity

37
Q

central giant cell granuloma - demographics

A
  • children and young adults
  • F > M
38
Q

central giant cell granuloma - location

A

anterior mandible (may cross midline, can occur in maxilla)

39
Q

central giant cell granuloma - clinical features

A
  • local swelling
  • pain
  • paresthesia - UNCOMMON
40
Q

central giant cell granuloma - radiographic features

A
  • root displacement or resorption
  • unilocular or multilocular
  • expansion without perforation of cortial plates
41
Q

central giant cell granuloma - histology

A

multinucleated giant cells (unique but not specific)

42
Q

central giant cell granuloma - differential diagnosis

A
  • Hyperparathyroidism “Brown Tumors”

indistinguishable from central giant cell granuloma

this is systemic

MUST differentiate before treatment!

  • Aneurysmal Bone Cyst - cavernous blood filled spaces
43
Q

central giant cell granuloma

A

curettage and complete excision

44
Q

cherubism

A

rare hereditary disease of mandible

45
Q

cherubism - demographics

A
  • children
  • M > F
46
Q

cherubism - preferred location

A

mandible

(bilateral rami; bilateral rami & posterior mandible; maxilla and madible)

47
Q

cherbuism - radiographic features

A

thinig of corticies

(easy to diagnose if in all for quadrants of MX & MN, otherwise may require genetic screening, thins the cortex but rarely penetrates)

48
Q

cherubism - treatment

A

slow regression after puberty, don’t over treat

may move teeth, but tends to reslove itself

radiotherapy contraindicated

49
Q

fibrous dysplasia

A

skeletal abnormaliy of poorly mineralized bone (bone never gets hard - spongiosum replaced with fibro-osseous tissue)

50
Q

two patterns of fibrous dysplasia

A
  1. monostotic
  2. polystotic
51
Q

monostotic fibrous dysplasia

A

one bone

52
Q

polystotic fibrous dysplasia

A

several bones

associated with Albright syndrome (several bones + endocrine abnormalities - may see precocious puberty)

53
Q

which form of fibrous dysplasia is most common

A

monostotic

54
Q

fibrous dysplasia - age

A

late childhood

become quiesent after puberty

55
Q

fibrous dysplasia - genetics

A

GNAS1 gene

56
Q

fibrous dysplasia - clinical findings

A
  • unilateral
  • painless
  • slow onset
57
Q

fibrous dysplasia - preferred location

A

mandible

58
Q

fibrous dysplasia - radiograpic features

A
  • eliptically shapped (unilaterally)
  • “gound glass,”“orange peel,” fuzzy appearance
  • diffuse, like “driven snow”
59
Q

fibrous dysplasia - histology

A

non-specific histology (requires combo of histo, radiology, and clinical findings)

osteoid and calcified bone (“Chinese characters”)

60
Q

fibrous dysplasia - prognosis

A

good

61
Q

fibrous dysplasia - treatment

A

monitor

commonly “burns out” around puberty

sugery only if it becomes obstructive

62
Q

fibrous dysplasia - differential diagnosis

A

ossifying fibroma

  • ossifying fibroma - the cortex is hard and has to be drilled through to get to lesion
  • fibrous dysplasia - the cotex is soft and can easily be cut through to get to lesion
63
Q

can you treat fibrous dysplasia with radiation therapy?

A

NO

radiation therapy is contraindicated

64
Q

osteoma

A

rare slow growing neoplasm of compact or cancellous bone

65
Q

osteoma - M?F

A

males

66
Q

what syndrome are osteomas associated with?

A

Gardner’s syndrome

(associated with multiple osteomas)

67
Q

osteoma - differential diagnosis

A
  • osteoblastoma
  • osteoid osteoma

these grow more rapidly, show more cellularity, and are associated with pain compared to osteomas

68
Q

do osteomas contain tooth remants/structures

A

yes

69
Q

osteoma - treatment

A

monitor and meaure risk

excsision if necessary (i.e. obstruction)

70
Q

exostoses/tori

A

not a neoplasm

reaction to bone stress

71
Q

ossifying fibroma

A

slow growing, well-demarcated, benign fibrou-osseous lesion of bone

72
Q

ossifying fibroma - origin

A

PDL cells

73
Q

ossifying fibroma - preferred location

A

mandible

(posterior to canine)

74
Q

aggressive form of ossifying fibroma

A

juvenile active ossifying fibroma

(ages 6-20, can grow very rapidly, need to monitor closely)

aggressive intervention required

75
Q

ossifying fibroma - M?F

A

females

76
Q

ossifying fibroma

A

lyctic lesion with radiopaque foci, usually has radiopaque border

looks like a marble

77
Q

ossifying fibroma - histology

A

mixture of fibrous and calcified tissue

“cementoid” calcifications

78
Q

ossifying fibroma - differential diagnosis

A

fibrous dysplasia

79
Q

ossifying fibroma - treatment

A

excision, curettage, enucleation

radiotherapy contraindicated

80
Q

osteoblastoma

A

rare, benign neoplasm associated with pain

(need to recognize that although incredibly rare if you are delaing with something that looks like an osteoma but is painful you could be dealing with this rare neoplasm)