Bone and Cartilage Tumors Flashcards Preview

Rheum/Musculoskeletal/Derm Week 1 > Bone and Cartilage Tumors > Flashcards

Flashcards in Bone and Cartilage Tumors Deck (78):
1

What bone conditions might show lesions in the brain?

Paget's disease or osteoma

2

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

Chondroblastoma or giant cell tumors

3

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

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

4

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

Enchondroma or Fibrous dysplasia

5

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

Ewing's sarcoma or Chondrosarcoma

6

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

Osteosarcoma or Juxtacortical osteosarcoma

7

What are the most common primary bone neoplasms?

-MM
-osteosarcoma
-chondrocytomas
-Ewing's sarcoma

Note that by far most bone tumors are metastatic carcinomas

8

Age rule with bone neoplasms

under 30= benign, over= malignant usually

9

Metastasis of bone neoplasms is usally via ____

venous circulation

10

Patient population for osteoma?

Adults and children (male slightly more than female)

11

Describe osteomas

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

12

Osteomas are associated with what?

Gardner syndrome

13

What is Gardner syndrome?

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

14

MOI of Gardner syndrome?

AD

15

What is gardner syndrome characterized by primarily?

multiple colon polyps

16

Where else can extracolonic tumors present in Gardner syndrome?

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

17

What case the epidermoid cysts seen in Gardner syndrome?

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

18

Describe osteoid osteomas

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

19

How does osteoid osteoma present?

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

20

How are osteoid osteomas treated?

radiofrequency ablation

21

Describe osteoblastomas.

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

22

How else are osteoblastomas differentiated from osteoid osteomas?

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

23

How are osteoblastomas treated?

curettage or excision

24

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

osteocarcomas

25

What patient population is common for osteosarcomas?

60% male, ages 10-20

26

Associations with osteocarcomas?

Paget disease and post-radiation in older patients

as well as Rb gene (retinoblastoma)= poor prognosis

27

Microscopic presentation of osteocarcoma?

spindle cell appearance with unmineralized/osteoid bone

28

How common is metastasis with osteocarcoma?

20% will have lung METs at diagnosis

29

Common spots for osteosarcoma?

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

30

Buzzword on xray with osterosarcoma?

Codman Triangle- periosteal reaction (indicates an aggressive tumor)

31

Buzzoword on bone biopsy with osteosarcoma?

'lace like'

32

5 yr survival of osteosarcoma?

about 75% in children and goes down with secondary disease

33

Acquired genetic abnormalites associated with increased risk of osteosarcoma?

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

What are the cartilage forming tumors?

-chondroma
-osteochondroma
-chondrosarcoma

35

What are the types of chondroma?

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

36

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

IDH1 and IDH2 (also seen in gliomas and acute myeloid leukemia (AML))

IDH= isocitrate dehydrogenase

37

What is the most common benign tumor of bone?

osteochondroma

38

Patient population for osteochondroma?

mostly males, 10-20 yrs old

39

Describe osteochondromas

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

40

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

broccili/califlower appearance budding off bone

41

Osteochondromas rarely transform to what?

chondrosarcomas (5-10%)

42

How are osterochondromas treated?

simple excision

43

Describe osteochondroma development

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

What covers the mushroom projection caused by osteochondromas?

a cap of cartilage (dont fucking forget this)

45

Describe enchondromas

usually asymptomatic tumor that presents in the small bones of hands and feet and are mostly solitary

treatment= excision or nothing

46

Patient population for enchondromas?

20-49, no gender preference

47

Note on multiple enchondromas

may produce sever deformities; associated with chondrosarcoma transformation

48

What is Maffuci syndrome?

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

49

What is Ollier disease?

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

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

F. Most regress with skeletal maturation

51

What are chondrosarcomas?

malignant cartilage forming tumors (NO OSTEOID)

52

Where do chondrosarcomas arise from?

the medulla of the pelvis or central/axial skeleton

53

Patient population for chondrosarcomas

men in 40s

54

What are the fibrous and Fibroosseous Tumors?

-Fibrous cortical defect
-Fibrous dysplasia

55

What are fibrous cortical defects?

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

56

Patient population for fibrous cortical defects?

teenagers, no gender preference

57

What causes fibrous cortical defects?

probably developmental defects

58

What causes fibrous dysplasia?

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

What bones are most commonly affected by fibrous dysplasia?

ribs and femur

60

Types of fibrous dysplasia?

-monosodic
-polysodic
-McCUne-Albright Syndrome

61

What is McCune-Albright Syndrome?

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

62

What are giant cell tumors of bone?

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

63

The neoplastic cells of giant cell tumors express what?

high levels of RANKL

64

Buzzword for giant cell tumors?

soap bubble on x-ray

65

What tumor is commonly described grossly as 'fish flesh'?

Ewing sarcoma (PNET)

66

What causes Ewing sarcoma?

malignant proliferation of poorly differentiated cells from NEUROECTODERM

second most common bone tumor in children after osteosarcoma

67

Where are Ewing sarcomas commonly found in bone?

diaphysis (medullary) in LONG bones

68

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

white male children under 15

69

X-ray appearance of Ewings?

onion skin

70

Common mutation of Ewing's?

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

71

How is Ewing's treated?

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

72

Prognosis for Ewing's?

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

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

T. Much

74

Common places that go to bone?

BLT with a Kosher Pickle

80% from breast, lung, and prostate

thyroid and kidney

75

Why would metastatic tumors go to axial bone?

it has a much richer blood supply

76

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

prostatic carcinoma

77

Which MET bone cancer is associated with lytic lesions?

MM (via icnreased osteoclast activation)

78

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

T. Except maybe lymphoma