Tumors of Bone and Cartilage (C-Hand) Flashcards

1
Q

Malignant tumors occurring in the diaphysis

A

Ewing’s sarcoma and chondrosarcoma

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2
Q

Benign tumors occurring in the diaphysis:

A

enchondroma and fibrous dysplasia

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3
Q

Malignant metaphysis lesions:

A

osteosarcoma and juxtacortical osteosarcomas

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4
Q

Benign conditions and tumors of metaphysis:

A
  1. non-ossifying fibroma
  2. osteoid osteoma
  3. giant cell tumors
  4. osteoblastoma
  5. chondromyxoid fibroma
  6. osteochondroma

(NO-GO-ChO)

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5
Q

Benign epiphysis lesions:

A

chondroblastoma and giant cell tumors

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6
Q

Fibrous dysplasia: 3 Patterns

A
  1. monostotic: single bone involved
  2. polyostotic: multiple bones involved
  3. McCune Albright syndrome: polyostotic, café-au-lait skin pigmentation, endocrinopathies
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7
Q

Fibrous dysplasia: histology

A

loose whorled pattern of fibroblastic tissue with irregular spicules of woven bone; May contain small islands of cartilage

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8
Q

Fibrous dysplasia: XR

A

large expansile medullary mass with lucent ground-glass appearance and well demarcated borders

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9
Q

Fibrous dysplasia is characterized by:

A

benign localized developmental arrest; all components of bone are present but do not mature

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10
Q

What pattern of fibrous dysplasia may transform to osteosarcoma?

A

polyostotic

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11
Q

Microscopically, what may be seen in Fibrous dysplasia?

A

poorly formed islets of bone (“Chinese character”)

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12
Q

Common location of fibrous dysplasia?

A

shoulder

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13
Q

Are fibrous cortical defects common?

A

yes: present in 30-50% of all kids >2y/o

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14
Q

Fibrous cortical defects: XR

A

“scooped out” lesion with dense borders surrounding it

**buzzowords = scalloped and sclerotic margins

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15
Q

Where fibrous cortical defects located?

A

lower femur and upper/lower tibia

**~1/2 are bilateral or multiple

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16
Q

What is the expected outcome of fibrous cortical defect?

A

spontaneous resolution

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17
Q

Fibrous cortical defects that are > 5-6 cm:

A

nonossifying fibromas

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18
Q

Epidemiology of fibrous cortical defects

A

Adolescents with few or no symptoms except pain (usually found incidentally on radiography)

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19
Q

Fibrous cortical defects: gross pathology

A

Eccentric, sharply delineated, metaphyseal lesions

Granular or dark red

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20
Q

Fibrous cortical defects: likely pathogenesis

A

developmental defect

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21
Q

Possible injury related to fibrous cortical defect?

A

fractures through thinned cortex

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22
Q

Non-ossifying fibroma: microscopically

A

woven mat or storiform pattern

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23
Q

Solitary bone cyst: pathogenesis

A

developmental abnormalities

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24
Q

Solitary bone cyst: Epidemiology + clinical presentation

A

Asymptomatic

Children and adolescents (usually male)

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25
Q

Solitary bone cysts: gross pathology

A

benign, fluid-filled, lytic bone lesions with thin bone margins

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26
Q

Solitary bone cysts: typical location

A

humerus or femur

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27
Q

Aneurysmal bone cysts: gross pathology

A

bloody, cystic lesions; “sponge filled with blood”

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28
Q

Aneurysmal Bone Cyst: location

A

surface of bone–usually in long bones and vertebral column (but may occur anywhere)

*(usually slow-growing, but may present with rapid expansion)

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29
Q

Aneurysmal Bone Cyst: microscopic pathology

A

rich capillary bed

**bleeding risk!!

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30
Q

Aneurysmal Bone Cyst: Epidemiology

A

Children and young adults

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31
Q

Benign tumors which may contribute to pathological fracture (3):

A

solitary bone cyst

aneurysmal bone cyst

fibrous cortical defect

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32
Q

When do Benign Primary Bone Neoplasms often present?

A

1st 3 decades of life

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33
Q

When are Benign Primary Bone Neoplasms removed?

A

if they cause pain

34
Q

How are Benign Primary Bone Neoplasms often discovered?

A

incidental XR

35
Q

What type of Benign Primary Bone Neoplasm may undergo malignant transformation?

A

chondromas

36
Q

How are Osteoma’s characterized?

A

round, slow-growing bone-forming tumors

37
Q

Osteoma: growth patterns

A
  1. project from sub- or endosteal surfaces of cortex
  2. Usually solitary (multiple = Gardner’s syndrome)
38
Q

Osteoid osteoma: clinical presentation + Epidemiology

A

Painful, generally < 2 cm lesion occuring most commonly in teens + twenty-something’s; more often in M > F (2:1)

39
Q

Osteoid osteoma: location

A
  1. cortical (rather than medullary)
  2. 50% = tibia and fibula
40
Q

What causes the main symptom of Osteoid osteoma? What makes it better/worse?

A

–Pain due to production of prostaglandin E2

–Worse: more commonly painful at night; Intake of alcohol >> massive increase in pain

–Better: aspirin

41
Q

Osteochondroma: synonyn

A

exostosis

42
Q

How are Osteochondroma characterized?

A

–benign cartilage capped tumors.

–tend to be attached to underlying skeletal system by a stalk

43
Q

What effects do Osteochondroma have on the skeletal system?

A

Displacement of growth plate in endochondral bones

44
Q

What is multiple hereditary exostosis?

A

autosomal dominant Osteochondroma, which may rarely give rise to chondrosarcomas

45
Q

How are chondroma’s characterized?

A

Benign tumor of hyaline cartilage

46
Q

Where do Enchondromas arise?

A

medullary cavity, often in tubular bones of hands and feet

**usually solitary

47
Q

Where do Juxtacortical chondromas arise?

A

on the surface of bones

48
Q

Nonhereditary syndrome characterized by formation of multiple enchondromas:

A

Ollier disease

49
Q

Syndrome characterized by formation of multiple enchondromas and soft tissue hemangiomas:

A

Maffucci syndrome

50
Q

Enchondroma: XR

A

Radiolucent nodules of hyaline cartilage + scalloped endosteal surface

51
Q

Giant Cell Tumor of Bone: Epidemiology

A

patients in their 20’s - 40’s

52
Q

Giant Cell Tumor of Bone: Primary lesion location? secondary lesion location?

A
  1. around knee
  2. bones of wrist
53
Q

How are Giant Cell Tumor of Bone characterized?

A

–benign, uncommon and locally aggressive

–Multinucleated osteoclast type giant cells

–commonly undergo cystic degeneratio

54
Q

Half of Malignant Bone Tumors are derived from:

A

blood forming cells and connective tissue cells

55
Q

Most common bone tumors?

A

Secondary (Metastatic)

56
Q

Sites from which metastases occur to the bone are characteristically:

A

Breast, prostate, lung, kidney and thyroid

57
Q

Most common primary malignant bone tumor?

A

Osteosarcoma

58
Q

Osteosarcoma: Clinical presentation + Epidemiology (typical)

A

painful lesions, typically in knee joint, in patients <20; often come to attention due to secondary injury (during sports, etc)

59
Q

Osteosarcoma occurs less frequently in what patient populations?

A

elderly, pt’s w/ Paget disease of bone or pt’s w/ hx of radiation

60
Q

Where does osteosarcoma present in older patients?

A

bones of the pelvis and the bones of the skull

61
Q

What conditions are osteosarcoma often associated with, clinically?

A

fractures, Retinoblastoma (Rb, MDM2), lung metastasis at diagnosis

62
Q

Osteosarcoma grows into:

A

soft tissue surrounding the bone

63
Q

What is the Codman triangle of periosteal elevation?

A

triangular shell of reactive bone formed by lifted periosteum; characteristic but not pathognomonic of osteosarcoma

64
Q

Chondrosarcoma: clinical presentation + Epidemiology

A

lesion often in axial skeleton, in patients ages 35-60

65
Q

Chondrosarcoma: location

A

Axial skeleton: trunk, pelvis, vertebrae, ribs (rarely involves extremities)
in diaphysis or metaphysis

66
Q

Second most common primary malignant tumor?

A

Chondrosarcoma

67
Q

Chondrosarcoma: treatment

A

Surgery (insensitive to chemotherapy)

68
Q

Chondrosarcoma may arise in association with:

A

a preexisting enchondroma

69
Q

Malignant bone neoplasms characteristically:

A

“do not respect joint spaces”

70
Q

Chondrosarcoma: microscopically

A

cells that mimic chondrocytes (“float” within ~chondroid matrix)

high nuclear cytoplasmic ration, nuclei and abundant mitotic figures

71
Q

Ewing Sarcoma: Epidemiology

A

White children, ages 10 to 15

72
Q

Ewing Sarcoma: microscopicaly

A

–very high nuclear cytoplasmic ratio (mostly nucleus) = “blue cell tumor”

–glycogen rich cells which tend to form small structures called Homer-Wright rosettes (tumor cells arranged in a circle around a central fibrillary space)

73
Q

85% of Ewing Sarcoma have genetic signatures, which show they are related to:

(What is the genetic signature?)

A

peripheral neuroectodermal tumors

t(11,22)+

74
Q

Ewing Sarcoma: gross pathology

A

onion skin appearance = layers of reactive periosteum and neoplasms

75
Q

Ewing Sarcoma: location

A

Arise in medulla, primarily in diaphysis of long bones

76
Q

Ewing Sarcoma: treatment and prognosis?

A

surgery and chemotherapy = 75% five year survival rate

77
Q

Most common tumors in bone?

A

mets

78
Q

In adults > 75% of skeletal metastasis originate from:

A

prostate, breast, lung, and kidney

79
Q

In children, skeletal metastasis originate from:

A

neuroblastoma, Wilms’ tumor, osteosarcoma, Ewing sarcoma (extraosseous) and rhabdomyosarcoma

80
Q

What is a common characteristic of metastatic bone tumors?

A

Usually multifocal, affecting marrow-rich areas (vertebrae, pelvis, ribs, skull and sternum)

81
Q

Metastatic Bone Tumors: XR

A

lytic, blastic or mixed lytic and blastic

82
Q

Metastatic Bone Tumors secrete:

A

substances that promote bone resorption