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System Pathology I -- Roecker > Exam 1 Pt2. Bone Tumors > Flashcards

Exam 1 Pt2. Bone Tumors Flashcards

1
Q

T/F. Bone tumors are most commonly malignant, asymptomatic, and slow growing.

A

False. Bone tumors are MC benign (asym. and slow growting), but can be malignant too

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2
Q

What are risk factors for low back pain of cancerous origin?

A
  • age 50 or older
  • LBP not relieved with rest or wakes one during night
  • Personal history of CA
  • LBP lasting longer than 1-month duration
  • Cachexia
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3
Q

What is the MC benign bone tumor?

A

osteochondroma

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4
Q

What are the three MC primary cancerous bone tumors?

A
  1. osteosarcoma
  2. chondrosarcoma
  3. Ewing sarcoma
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5
Q

In what ages are bone tumors MC benign? What about malignant?

A

if develop 0-30, MC benign, if develop in older adults, MC malignant

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6
Q

What are some risk factors for Bone tumors?

A
  • mutations in RB or TP53 genes

- inflammation (AVN, hardware, etc.)

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7
Q

Where are bone tumors MC?

A

long bones of extremeties

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8
Q

If a bone tumor is cancerous what are some symptoms?

A

fever, fatigue, cachexia

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9
Q

What are two very important aspects to take into consideration when narrowing down the type of bone tumor?

A

Age and location, also radiographic appearance

Ex: age 10-20 at knee = osteosarcoma

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10
Q

T/F. Bone marrow is a common site for cancer mets; therefore, when cancer is discovered within the bone, more likely to be cancer that mets to bone.

A

True

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11
Q

What are the three ways we are categorizing bone tumors?

A
  1. Bone-forming tumors
  2. Cartilage forming tumors
  3. Tumors of unknown origin
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12
Q

_______ will produce bony tissue with variable levels of mineralization, most likley with poorly-mineralized osteoid and mineralized woven bone.

A

Bone-forming tumors

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13
Q

T/F. Osteoid Osteoma and Osteoblastoma are both malignant.

A

False, they are both BENIGN, but differ in size

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14
Q

What type of bone-forming tumor is benign and smaller than 2cm and create a “nest-like” appearance?

A

Osteoid osteomas

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15
Q

What sex and age group is most likely to get an osteoid osteoma?

A

males 2x likely; ages 10-20

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16
Q

T/F. Osteoid osteomas are round/oval shape are pain is not localized and not relieved by aspirin.

A

False, yes, they are oval shape, BUT the pain is well-localized and IS relieved by aspirin

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17
Q

Where do Osteoid osteomas MC develop?

A

cortex of bones in lower extremities; metaphysis of femur and tibia

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18
Q

What is an osteoblastoma?

A

a benign, bone-forming tumor, at least 2cm are larger

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19
Q

Where are osteoblastoma MC located and what type of pain occurs?

A

MC in vertebral column–locally destructive; and poorly-localized pain that is Unrelieved by aspirin

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20
Q

What age range are osteoblastomas MC? Whats the Tx?

A

MC ages are 10-20 years (same as osteoid osteomas); Tx as if cancer due to dangerous area on spine…excision

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21
Q

What is a malignant, bone-forming tumor that commonly occurs ages 10-20?

A

osteosarcoma; 75% of all cases occur prior to age 20

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22
Q

If osteosarcoma occurs in an adult, what are the likely the possible causes?

A

= “secondary”; >40 years

  • Paget Disease
  • AVN
  • ionizing radiation
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23
Q

T/F. Females make up about 60% all cases of osteosarcomas.

A

false, males make up the 60%

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24
Q

Where are osteosarcomas MC in the body?

A

metaphyseal region of long bones in extremities

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25
Q

When an ostoesarcoma develops in a characteristic adolescent, where is the location?

A

ages 10-20 yrs; within metaphyseal region of distal femur or proximal tibia = ~60% all osteosarcomas

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26
Q

What are other common locations of osteosarcomas (other than knee)?

A
  • proximal femur/pelvis (15%)
  • proximal humerus (10%)
  • mandible or facial bones (8%)
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27
Q

What type of pain is experienced with osteosarcomas?

A

painful tumors; increase pain freq, and pain intensity as tumor enlarges and invades

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28
Q

What is the MC place of osteosaroma to mets to?

A

lungs; pulmonary mets is present in ~20% of osteosarcomas when initially diagnosed

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29
Q

What are the x-ray appearances of osteosarcomas?

A
  • Codman Triangle
  • large bony lesion with destructive appearance/ cortical destruction, “starburst”
  • osteosclerotic (brighter white) over osteolysis (darker regions) = “mixed” lytic
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30
Q

When osteosarcomas start to invade where do they “exit/invade”?

A

the cortex of bone–> called periosteal reaction

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31
Q

What are periosteal reactions? What does one assume when they see one?

A

they develop following periosteal irritation from non-invasive or invasive lesions of bone; assume aggressive CA, until proven otherwise

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32
Q

Describe the appearance of Codman triangle on an x-ray. What is an indication we can assume from seeing this on an x-ray?

A

raised triangular “shadow” between the cortex of the bone and original cortical bone; Indicates the tumor is invading through the cortex and is behaving aggressively

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33
Q

What gene mutations are common in someone who has osteosarcoma? What will increase an inds. risk by 1000x of getting osteosarcoma?

A 
RB and TP53 mutations (tumor suppressor genes); 
retinoblastoma syndrome (cancer in retina)
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34
Q

Individuals over 40 who get osteosarcoma, how is there survival rate?

A

typically it is lethal

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35
Q

What are the three main Cartilage-Forming Bone tumors?

A
  1. Osteochondroma
  2. Enchondroma
  3. chondrosarcoma
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36
Q

Which is MC, benign or malignant cartilage-forming bone tumors?

A

benign

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37
Q

What is a cartilage-bone forming tumor that is benign and also called “exostosis”, typically projecting out from bony cortex with a cartilaginous cap?

A

osteochonroma

(exostosis = “sticks out”

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38
Q

Osteochondromas can manifest in two ways. What are those two ways?

A

pedunculated mass = narrow base

sessile mass = broad base

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39
Q

Are osteochondromas MC inherited or sporadic?

A
  • MC sporadic (85%)

- inherited (15%) and involve multiple tumors

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40
Q

What are the two ways that cartilage-bone forming tumors produce when they grow?

A

bony hyaline cartilage = clear, glass-like
OR
myxoid cartilage = mucus like

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41
Q

What is it called when an individual has multiple osteochondromas and it is inherited?

A

Multiple hereditary exostoses

- (usually mutated EXT1 or EXT2)–> dyregulated cartilage growth

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42
Q

What are the chances that osteochondroma transitions into cancer? What cancer would it transition into?

A

rare; <1% transition risk into chondrosarcoma

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43
Q

Where are osteochondromas discovered?

A

in metaphyseal region, near growth plate in long bones of lower extremities; project from cortex

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44
Q

What is the MC place for an osteochondroma to manifest? Where else can it manifest?

A

MC at the knee

- also as pelvis, scapula, ribs, hand/feet

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45
Q

What is the MC age for osteochondromas to be discovered?

A

ages 10-30; adolescent or young adulthood

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46
Q

T/F. Osteochondromas are usually very painful.

A

False, they are usually asymptomatic unless mass Fx, compresses soft tissue, or can be palpated through skin

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47
Q

Are osteochondromas MC in males of females?

A

males, 3-1 ratio

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48
Q

What type of benign cartilage-forming tumor manifests as a solitary radiolucent lesion within tubular bones of hands and feet?

A

enchondroma (chondroma)

49
Q

When are enchondromas typically diagnosed?

A

ages 20-50; therefore is a benign tumor of adulthood

50
Q

Are enchondromas MC in males or females?

A

2x as likely in males

51
Q

Where do enchondromas MC manifest?

A

in proximal phalanges (40-50%) and metacarpals (305)

52
Q

How are enchondromas diagnosed?

A

typically asymptomatic and detected incidentally; larger ones may weaken bone and cause pathological Fx

53
Q

What type of bone tumor manifests with “nodules” of bluish-white hyaline cartilage with well-circumscribed lesions?

A

enchondromas

54
Q

What is the then zone surrounding an enchondroma’s circumscribed lesions called?

A

O-ring sign

55
Q

What is the central site of an enchondroma likely to form?

A

AVN, may cause irregular areas of opacification due to stimulated dystrophic calcification

56
Q

What is the management of enchondromas?

A
  • smaller, asymptomatic ones = no Tx yet

- larger or ones that caused pathological Fx = are removed

57
Q

What is Ollier Disease? How common is it?

A

(enchondromatosis); involves formation of multiple large enchondromas, casing severe bony deformation; rare at 1 in 100,000 inds.

58
Q

Enchondromas are named based on location on bone. What are the two ways? Which is rare?

A

Enchondroma = medullary

Juxtacortical chondroma = cortical; RARE

59
Q

What is the 2nd MC primary bone cancer?

A

chondrosarcoma

MC is osteosarcoma

60
Q

X-ray of a 50 year old man with a cartilage-forming bone tumor in proximal humerus that has a “popcorn” appearance. What are you thinking it is?

A

chondrosarcoma

61
Q

What age group are chondrosarcomas most likely found in? Which sex?

A

ages 40-60

men 2x more likely

62
Q

What type of cartilage do chondrosarcomas produce?

A

hyaline and myxoid cartilage

63
Q

Where are chondrosarcomas most likely to develop?

A

bones of pelvis, proximal femur, proximal humerus, sacpula, ribs, sternum
(RARE to develop in distal extremities)

64
Q

Radiographically, what tumor has “stippled” or “popcorn” calcifications?

A

chondrosarcoma

65
Q

What does gross inspection of a chondrosarcoma look like?

A

large mass with a “glistening gray-white” or “translucent” cartilage

66
Q

T/F. Chondrosarcomas MC develop Juxtacortical.

A

False. MC develop intramedullary

67
Q

Are low-grade or high-grade chondrosarcomas more common? Describe it.

A

low-grade are MC; smaller, slow-growing (indolent) and thicken surrounding cortical bone

68
Q

What is the prognosis for low-grade and high-grade chondrosarcomas?

A

low-grade = favorable, 90% 5 year survivability

high-grade = poor prognosis, 40% 5 year survivability

69
Q

A chondrosarcoma on an x-ray that appears to have increase radiolucency will increase the chance of it being high or low-grade? Why?

A

high-grade, because eroding away the cortex

low-grade thicken cortical bone

70
Q

Where is the MC place a high-grade chondrosarcoma will mets to? Via what way?

A

MC to lungs
or to other bone
via invasion of surrounding medullary structures or hematogenously

71
Q

Which cartilage-forming tumor is more likely to destroy a joint?

A

chondrosarcoma

72
Q

What are the bony lesions called that have been described as benign tumors of neoplastic fibroblasts or macrophages? What are they being described as more recently?

A

Fibrous cortical defects (FCD) and Nonossifying fribromas (NOF)

more of a reaction to trauma at the area of the periosteal/cortical reaction

73
Q

What part of bone are Fibrous cortical defects (FCD) and Nonossifying fribromas (NOF) most likely to involve?

A

metaphysis or diaphysis of lower extremity near cortex

  • tibia or femur (MC at knee)
74
Q

How common are Fibrous cortical defects (FCD) and Nonossifying fribromas (NOF)?

A

very common, may be found in up to 40% of skeletally immature children

75
Q

What is the difference between Fibrous cortical defects (FCD) and Nonossifying fribromas (NOF) ?

A

they are bony lesions that are histologically identical

FCD = smaller
NOF = larger (>3cm)
76
Q

What is the x-ray appearance of Fibrous cortical defects (FCD) and Nonossifying fribromas (NOF)?

A

well-defined radiolucent lesion with thin sclerosis

77
Q

What can a larger Nonossifying fribromas (NOF) cause?

A

predispose ind to pathologic fx, by creating a large area of structurally-weak bone

78
Q

What is a non-neoplastic alteration in bone, and is localized bony differentiation, commonly called “arrested development” bone?

A

Fibrous Dysplasia (3 clinical manifestations)

79
Q

What does Fibrous Dysplasia involve?

A

involves replacing normal bone with fibrous stroma and areas of irregular woven bone

80
Q

What do all three cases of Fibrous Dysplasia originate from?

A

a congenital embryological GNAS gene mutation, spontaneously

81
Q

What are the three ways Fibrous Dysplasia can clinically manifest?

A
  1. Monostotic Fibrous Dysplasia (MC)
  2. Polyostotic Fibrous Dysplasia
  3. McCune- Albright Syndrome
82
Q

What is the MC type of Fibrous dysplasia?

A

Monostotic fibrous dysplasia (~70%)

83
Q

When is Monostotic fibrous dysplasia usually discovered? Bones most likely involved?

A

10-30 years old; asymptomatic, little bony distortion

ribs, femur, calvaria, mandible, or humerus

84
Q

What does Polyostotic fibrous dysplasia usually manifest like?

A
  • multiple bones
  • more severe than monostotic FD
  • severe bony deformation (enlargement)
85
Q

What type of FD radiographically appears as a “ground glass” appearance, and may have osseous distortion described as a “shepherd crook”?

A

Polyostotic fibrous dysplasia

86
Q

What bones is Polyostotic fibrous dysplasia likely to involve?

A
  • craniofacial bones (50% cases)
  • proximal femur
  • pelvis
  • humerus
  • tibia
87
Q

What age is Polyostotic fibrous dysplasia most likely detected?

A

late childhood/ adolescence

88
Q

If someone manifests with polyostotic fibrous dysplasia, endocrine hyperfunctioning, and cafe au lait skin lesions, what do they have?

A

McCune-Albright Syndrome

least common of FD at 3%

89
Q

T/F. The polyostotic fibrous dysplasia that manifests in McCune-Albright Syndrome is usually more severe than when it manifests as just Polyostotic fibrous dysplasia alone.

A

False, it is usually less severe

90
Q

What may the endocrine hyperfunctioning caused in McCune-Albright syndrome develop from?

A
  • pituitary adenoma
  • adrenal hyperplasia
  • hyperplasia of thyroid gland
    (is early-onset puberty)
91
Q

Describe the appearance of the cafe au lait skin lesions ass. with McCune-Albright syndrome.

A
  • commonly unilateral (on one side of body)
  • fairly large
  • “coast of Maine”/ irreg. jagged boarders
92
Q

What is the 2nd MC pediatric bone cancer?

What percentage does it represent of all bone cancers?

A

Ewing sarcoma

10%

93
Q

What is a malignant primary bone tumor composed of primitive (undifferentiated) “small round cells”?

A

Ewing sarcoma

94
Q

What cancer MC affects ages 10-20, males, 9x more likely in causasians, and appears at diaphysis of extremity long bones?

A

Ewing sarcoma

95
Q

If one has bone cancer that mimics symptoms of an infection and is a painful and enlarging mass, what do we think it could be?

A

Ewing sarcoma

  • area is warm, tender, swollen
  • fever
  • increase WBCs
  • elevated ESR; “sed rate”
96
Q

What type of bone cancer has a characteristic “onion-skin” appearance?

A

Ewing sarcoma

97
Q

Where is Ewing sarcoma most likely to originate from? What does it “do” to bone?

A

medullary cavity of bone and freq. causes osteolytic area of destruction; that may invade surrounding soft tissues

98
Q

What type of radiographic appearance can Ewing sarcoma have that mimics osteosarcoma? How to tell them apart?

A

“sunburst” appearance

Ewing sarcoma– diaphysis
Osteosarcoma– metaphyseal region

99
Q

T/F. Ewing sarcomas are usually aggressive and treated with surgical excision and chemo.

A

True

100
Q

Ewing sarcomas has a _____ 5 year survival, and a ____ complete remission.

A

75% 5 year survival

50% complete remisison

101
Q

What is another bone tumor that is nearly identical to Ewing sarcomas, except the cells that compose it contain a degree of what?

A

Primitive Neuroectodermal Tumor (PNET)

  • cells have some degree of neuroectodermal differentiation and manifest with Homer-Wright (pseudo) rosettes
102
Q

What bone tumor is an intermediate tumor, not clearly benign or malignant?

A

Giant Cell tumor of Bone

103
Q

What bone tumor is named for the presence of multinucleated “giant cells”? How does it manifest?

A

Giant Cell tumor of Bone

  • locally invasive
  • create large area of osteolysis
  • freq. cause bony expansion/distortion
104
Q

What do Giant Cell tumors of bone typicallly occur?

A

at the metaphyseal and epiphyseal areas of long bones in extremities

105
Q

What age group are Giant Cell tumors of bone exclusive to? What are the common locations of manifestation?

A

adults– ages 20-40

MC at knee

  • distal femur
  • prox. tibia
  • distal radius
  • sacrum
  • proximal humerus
106
Q

Radiographically, what bone tumor manifests as an isolated osteolytic mass that is eccentric of an extremity long bone?

A

Giant Cell tumor of bone

107
Q

What radiographically appearance is typically of Giant Cell tumors of bone?

A

thin “shell of reactive bone” surrounding bony lesion and freq/ destroy cortical bone and cause bulging tissue mass

“soap-bubble” appearance

108
Q

Gaint cell tumors of bone MC develop near a joint, so what do they cause?

A
  • decrease ROM
  • arthritis-like pain
  • pathological fx
109
Q

What percentage of Giant cell tumors of bone return following curettage?
What percent mets and to where?

A

50% return

2-4% mets to lungs

110
Q

Cancer in the bone is most commonly due to what?

A

metastasis (mets to bone is MC than primary bone CA)

111
Q

What is the most common area for cancer to mets in bone?

A

spine

112
Q

Of the cancer that mets to bone, which ones make up over 75% of adult bony mets?

A
  • prostate
  • breast
  • kidney
  • lung
113
Q

T/F. Skeletal mets is most likely to affect a single bone.

A

False; most likely to be multifocal (multiple sites)

114
Q

Presence of secondary bone tumor indicates what about condition of ind.?

A

very poor prognosis; cancer in advanced stage (stage IV)

115
Q

What are the three ways skeletal mets may appear? Which is MC?

A
  1. osteolytic (dark)
  2. osteoblastic (whitish)
  3. “mixed” lytic and blastic (MC)
116
Q

What is indicative of these cancers when they mets to bone:

  1. prostate
  2. breast
  3. lung
A
  1. osteoblastic (hence ivory vert. sign)
  2. osteolytic
  3. “mixed” osteolytic/osteoblastic
117
Q

What cancer is most commonly ass. with skeletal mets in children?

A
  • neuroblastoma
  • Wilms tumor
  • osteosarcoma
  • rhabdomyosarcoma
  • Ewing sarcoma
118
Q

What are “red flags” for low back pain of cancerous origin?

A
  1. > 50 years old
  2. history of CA
  3. cachexia
  4. LBP unrelieved with rest
  5. pain lasting over a month
  6. failure to improve after a month of care
119
Q

What is the sensitivity and specificity for an ind. what is older than 50, has history of CA, and has cachexia, to have skeletal mets?

A

100% sensitive

60% specific

System Pathology I -- Roecker (14 decks)

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