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STEP Pathology > deck_5367042 > Flashcards

deck_5367042 Flashcards

1
Q

What bone conditions might show lesions in the brain?

A

Paget’s disease or osteoma

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2
Q

What benign bone neoplasms can present on the epiphysis of a bone?

A

Chondroblastoma or giant cell tumors

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3
Q

What benign bone neoplasms can present on the metaphysis of long bones?

A

-osteoblastoma-osteochondroma-Non-ossifying fibroma-osteoid osteoma-Chondromyxoid fibroma-Giant cell tumor

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4
Q

What benign bone neoplasms can present on the diaphysis of long bones?

A

Enchondroma or Fibrous dysplasia

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5
Q

What malignant bone neoplasma can present on the diaphysis of long bone?

A

Ewing’s sarcoma or Chondrosarcoma

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6
Q

What malignant bone neoplasma can present on the metaphysis of long bones?

A

Osteosarcoma or Juxtacortical osteosarcoma

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7
Q

What are the most common primary bone neoplasms?

A

-MM-osteosarcoma-chondrocytomas-Ewing’s sarcoma Note that by far most bone tumors are metastatic carcinomas

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8
Q

Age rule with bone neoplasms

A

under 30= benign, over= malignant usually

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9
Q

Metastasis of bone neoplasms is usally via ____

A

venous circulation

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10
Q

Patient population for osteoma?

A

Adults and children (male slightly more than female)

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11
Q

Describe osteomas

A

bone formaing tumors composed of compact or mature trabecular bone that are confined almsot exclusively to cranial-facial bones (paranasal sinuses) and present with pain, headache, and vision changes

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12
Q

Osteomas are associated with what?

A

Gardner syndrome

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13
Q

What is Gardner syndrome?

A

a variant of familial adenomatous polyposis caused by APC gene mutations on chromosome 5q21

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14
Q

MOI of Gardner syndrome?

A

AD

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15
Q

What is gardner syndrome characterized by primarily?

A

multiple colon polyps

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16
Q

Where else can extracolonic tumors present in Gardner syndrome?

A

-osteomas in the skull-thyroid cancers-epidermoid cysts-fibromas-desmoid tumors in approx. 15% of patient s

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17
Q

What case the epidermoid cysts seen in Gardner syndrome?

A

doubling of the squamous layer of skin with keratin debris in middle

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18
Q

Describe osteoid osteomas

A

benign tumors of young (less than 25) males associated with osteoblasts that produce osteoid surrounded by a rim of reactive bone (common in cortex of long bones of the leg)less than 1.5cm in diameter

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19
Q

How does osteoid osteoma present?

A

(may be nocturnal) bone pain in the leg of a young male that resolves with aspirin

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20
Q

How are osteoid osteomas treated?

A

radiofrequency ablation

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21
Q

Describe osteoblastomas.

A

similiar to an osteoid osteoma but is LARGER (2+ cm), and is associated with dull bone pain but IS NOT relieved by aspirin

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22
Q

How else are osteoblastomas differentiated from osteoid osteomas?

A

more commonly found in the VERTEBRAE, in female and children, and NOT relieved by aspirin

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23
Q

How are osteoblastomas treated?

A

curettage or excision

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24
Q

What bone neoplasm is characterized by a MALIGNANT proliferation of osteoblasts?

A

osteocarcomas

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25
Q

What patient population is common for osteosarcomas?

A

60% male, ages 10-20

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26
Q

Associations with osteocarcomas?

A

Paget disease and post-radiation in older patients as well as Rb gene (retinoblastoma)= poor prognosis

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27
Q

Microscopic presentation of osteocarcoma?

A

spindle cell appearance with unmineralized/osteoid bone

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28
Q

How common is metastasis with osteocarcoma?

A

20% will have lung METs at diagnosis

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29
Q

Common spots for osteosarcoma?

A

-knee metaphysis (60%)-hip (15%)-shoulder (10%)-nasal (8%)

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30
Q

Buzzword on xray with osterosarcoma?

A

Codman Triangle- periosteal reaction (indicates an aggressive tumor)

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31
Q

Buzzoword on bone biopsy with osteosarcoma?

A

‘lace like’

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32
Q

5 yr survival of osteosarcoma?

A

about 75% in children and goes down with secondary disease

33
Q

Acquired genetic abnormalites associated with increased risk of osteosarcoma?

A

Rb, a critical negative regulator of the cells (mutation= 1000x risk for OS.TP53 mutation (li-Fraumeni syndrome or sporadic)INK4a, (incodes tumor suppressors)MDM2 and CDK4, cell cycle regulators that inhibit p53 and RB function, respectively

34
Q

What are the cartilage forming tumors?

A

-chondroma-osteochondroma-chondrosarcoma

35
Q

What are the types of chondroma?

A

benign cartilaginous tumor that is classified as either:1) enchondroma (arise from diaphyseal medullary cavity)2) subperiosteal/juxtacortical chondroma3) soft tissue chondroma

36
Q

What genes have been ID’d in the chondrocytes if syndromic and solitary enchondromas?

A

IDH1 and IDH2 (also seen in gliomas and acute myeloid leukemia (AML))IDH= isocitrate dehydrogenase

37
Q

What is the most common benign tumor of bone?

A

osteochondroma

38
Q

Patient population for osteochondroma?

A

mostly males, 10-20 yrs old

39
Q

Describe osteochondromas

A

slow growing, painful benign tumors that usually stop growing at puberty and ossify

40
Q

What characteristic appearance do osteochondromas take on in x-ray?

A

broccili/califlower appearance budding off bone

41
Q

Osteochondromas rarely transform to what?

A

chondrosarcomas (5-10%)

42
Q

How are osterochondromas treated?

A

simple excision

43
Q

Describe osteochondroma development

A

intitially, at the epiphyseal plate, there is a small outpouching of cartilage, which grows proximally to the epiphyseal plate and comes to resemble a mushroom/califlower growing out of bone

44
Q

What covers the mushroom projection caused by osteochondromas?

A

a cap of cartilage (dont fucking forget this)

45
Q

Describe enchondromas

A

usually asymptomatic tumor that presents in the small bones of hands and feet and are mostly solitary treatment= excision or nothing

46
Q

Patient population for enchondromas?

A

20-49, no gender preference

47
Q

Note on multiple enchondromas

A

may produce sever deformities; associated with chondrosarcoma transformation

48
Q

What is Maffuci syndrome?

A

multiple enchondromas and soft tissue hemangiomas assoicated with ovarian carcinoma and brain gliomas

49
Q

What is Ollier disease?

A

nonhereditary disease of multiple enchondromas of LONG bones and flat bones (up to 50% of skeleton) with associated skeletal deformities and often ovarian sexcord tumors

50
Q

T or F. Most lesions associated with Ollier disease progress into adulthood

A

F. Most regress with skeletal maturation

51
Q

What are chondrosarcomas?

A

malignant cartilage forming tumors (NO OSTEOID)

52
Q

Where do chondrosarcomas arise from?

A

the medulla of the pelvis or central/axial skeleton

53
Q

Patient population for chondrosarcomas

A

men in 40s

54
Q

What are the fibrous and Fibroosseous Tumors?

A

-Fibrous cortical defect-Fibrous dysplasia

55
Q

What are fibrous cortical defects?

A

NON-OSSIFYING fibromas greater than 5cm with an intramedullary component that is benign and typically asymptomatic

56
Q

Patient population for fibrous cortical defects?

A

teenagers, no gender preference

57
Q

What causes fibrous cortical defects?

A

probably developmental defects

58
Q

What causes fibrous dysplasia?

A

benign tumor arising during development forming a intramedullary fibrous mass (fibrous tissue instead of medullary bone) resulting in painful swollen bones prone to fracture that presents in early adolescence

59
Q

What bones are most commonly affected by fibrous dysplasia?

A

ribs and femur

60
Q

Types of fibrous dysplasia?

A

-monosodic-polysodic-McCUne-Albright Syndrome

61
Q

What is McCune-Albright Syndrome?

A

fibrous dysplasia plus cafe-au-lait skin pigmentations and endocrine abnormalities, especially precocoius puberty

62
Q

What are giant cell tumors of bone?

A

tumors of young adults (20-40s) arising from the epiphysis of long bones, typically the knee

63
Q

The neoplastic cells of giant cell tumors express what?

A

high levels of RANKL

64
Q

Buzzword for giant cell tumors?

A

soap bubble on x-ray

65
Q

What tumor is commonly described grossly as ‘fish flesh’?

A

Ewing sarcoma (PNET)

66
Q

What causes Ewing sarcoma?

A

malignant proliferation of poorly differentiated cells from NEUROECTODERM second most common bone tumor in children after osteosarcoma

67
Q

Where are Ewing sarcomas commonly found in bone?

A

diaphysis (medullary) in LONG bones

68
Q

Patient population of Primitive neuroectodermal tumor (PNET) (aka Ewing)?

A

white male children under 15

69
Q

X-ray appearance of Ewings?

A

onion skin

70
Q

Common mutation of Ewing’s?

A

t(11,22) translocation (EWS-FLI1 fusion)

71
Q

How is Ewing’s treated?

A

aggressive treated with neoadjuvant chemo followed by surgical excision with or without radiation

72
Q

Prognosis for Ewing’s?

A

effective dchemo achieves 5-yr survival of 75% and long term cure of 50%the amount of chemo-induced necrosis is an important prognostic finding

73
Q

T or F. Metastatic bone tumors are more common than primary

A

T. Much

74
Q

Common places that go to bone?

A

BLT with a Kosher Pickle80% from breast, lung, and prostatethyroid and kidney

75
Q

Why would metastatic tumors go to axial bone?

A

it has a much richer blood supply

76
Q

Which MET bone cancer is associated with osteoblastic (bone forming) lesions?

A

prostatic carcinoma

77
Q

Which MET bone cancer is associated with lytic lesions?

A

MM (via icnreased osteoclast activation)

78
Q

T or F. All MET bone cancers are Stage IV with poor prognosis

A

T. Except maybe lymphoma

STEP Pathology (80 decks)

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