Bone tumors

Question 1

What are the four pieces of information critical in diagnosis of a mass in bone?

Answer 1

Age, sex, location, radiographic appearance (NOT histo)

Question 2

What malignant tumors occur in the diaphysis?

Answer 2

Ewing’s sarcoma, chondrosarcoma

Question 3

What benign tumors occur in the diaphysis?

Answer 3

Endosarcoma, fibrous dysplasia

Question 4

What malignant tumors occur in the metaphysis?

Answer 4

osteosarcoma, juxtacortical osteosarcroma

Question 5

What benign tumors occur in the metaphysis?

Answer 5

osteoblastoma, osteochondroma, non-ossifying fibroma, osteoid osteoma, chondromyxoid fibroma, giant cell tumors

Question 6

What benign tumors occur in the epiphysis?

Answer 6

Chondroblastoma and giant cell tumors

Question 7

What are the 3 distinctive fibrous dysplasia patterns?

Answer 7

monostotic: single bone
polyostotic: multiple bones
McCune Albright syndrome: polyostotic, cafe au lait skin pigmentation, endocrinopathies

Question 8

What kind of fibrous dysplasia may transform to osteosarcoma?

Answer 8

polyostotic disease

Question 9

_____ is a benign lesion with localized developmental arrest. All components on bone are present but do not mature.

Answer 9

Fibrous dysplasia

Question 10

Where in the bone is fibrous dysplasia usually seen?

Answer 10

Diaphysis

Question 11

How does fibrous dysplasia look on x-ray?

Answer 11

ground-glass lucent appearance with well-demarcated borders. Can appear moth-eaten

Question 12

Describe the histology of fibrous dysplasia.

Answer 12

Loose, whorled “Chinese Characters” pattern of FIBROBLASTIC tissue with irregular spicules of bone. Lack osteoblastic activity–> lots of collagen. Can undergo cystic degeneration.

Question 13

How common are fibrous cortical defects?

Answer 13

Very- 30-50% of all children less than 2 y/o have these.

Question 14

Where in the bone are fibrous cortical defects seen?

Answer 14

metaphysis of femur and proximal tibia

Question 15

How many lesions are present with fibrous cortical defects?

Answer 15

1/2 are bilateral or multiple

Question 16

What is a nonossifying fibroma?

Answer 16

A fibrous cortical defect that is bigger than 5-6 cm

Question 17

How does fibrous cortical defect look in X-ray? What age is it usually presented in?

Answer 17

“Ice cream scoops” eccentric, sharply delineated, metaphyseal lesion with dense sclerotic borders. Usually adolescents.

Question 18

Clinical features of fibrous cortical defect?

Answer 18

Seen in adolescents, few or no symptoms except pain, usually found incidentally on xray, fractures can occur through thinned cortex

Question 19

Gross pathology of fibrous cortical defect

Answer 19

granular and brown or dark red, eccentric, sharply delimited

Question 20

What is the histological appearance of nonossifying fibroma?

Answer 20

Storiform- woven mat

Question 21

Describe solitary bone cysts.

Answer 21

benign, fluid-filled cyst, thing lytic bone lesion,that is not a true neoplasm but a growth abnormality and is usually asymptomatic.

Question 22

Where are solitary bone cysts usually seen?

Answer 22

humerus or femur (2/3)

Question 23

Who do solitary bone cysts usually occur in?

Answer 23

children and adolescents, usually male

Question 24

Symptoms of solitary bone cysts

Answer 24

usually asymptomatic but can contribute to pathological fracture

Question 25

____ are very bloody, cystic lesions that appear like a "sponge filled with blood" that can contribute to fractures.

Answer 25

aneurysmal bone cyst

Question 26

What does an aneurysmal bone cyst look like histologically?

Answer 26

Clotted blood and highly vascularized tissue. Giant cells could be present as well.

Question 27

T or F. Aneursymal bone cyst is a true tumor.

Answer 27

F

Question 28

Where do aneurysmal bone cysts usually arise?

Answer 28

Arise from surface of the bone. Long bones and vertebral column

Question 29

What age group can present with aneurysmal bone cysts?

Answer 29

children and young adults

Question 30

How fast to aneursymal bone cysts grow?

Answer 30

usually slow, but can rapidly expand

Question 31

What may be encountered in surgery with aneurysmal bone cysts?

Answer 31

brisk bleeding.

Question 32

What are the benign primary bone neoplasms?

Answer 32

osteoma, osteoid osteoma, osteochondroma/chondroma, fibroma, giant cell tumor

Question 33

When is the greatest frequency of benign primary bone neoplasms?

Answer 33

first3 decades of life

Question 34

Do benign primary bone neoplasms undergo malignant transformation?

Answer 34

rarely EXCEPT FOR CHONDROMA--> CHONDROSARCOMA

Question 35

Describe osteoma.

Answer 35

Benign primary bone neoplasm. Bone forming tumor. Round tumors that project from sub- or endosteal surfaces of cortex. Slow growing usually of little significance unless they cause obstruction

Question 36

Describe the number of lesions seen in osteoma.

Answer 36

usually solitary. multple seen in Garnder's syndrome which involves GI tract

Question 37

____ is a benign primary bone neoplasm of osteoblasts.

Answer 37

osteoid osteoma

Question 38

Size of osteoid osteoma lesion

Answer 38

less than 2 cm

Question 39

Age group presenting with osteoid osteoma

Answer 39

teens and twenties

Question 40

Location of osteoid osteoma

Answer 40

50% involve tibia and fibula, usually cortical.

Question 41

Describe symptoms associated with osteoid osteoma.

Answer 41

Painful- produce PGE2 and have characteristic pattern of pain. They are more commonly painful at night and they are relieved by aspirin. Intake of alcohol causes a massive increase in pain.

Question 42

Sex predominance of osteoid osteoma

Answer 42

M 2:1

Question 43

Describe osteochondroma

Answer 43

benign, cartilage capped tumor that is attached to underlying skeleton by a stalk. Involves displacement of growth plate in endochondral bone.

Question 44

What genetic condition exists with osteochondroma

Answer 44

Mutliple hereditary exostosis. AD that rarely gives rise to chondrosarcoma

Question 45

What do osteochondromas look like on x-ray

Answer 45

cauliflower at epiphyseal plate

Question 46

___ are benign tumors of hyaline cartilate

Answer 46

Chondroma

Question 47

What kind of chondroma arises in medullary cavity of tubular bones like hand and feet?

Answer 47

enchondroma

Question 48

What kind of chondroma arises on the surface of bones?

Answer 48

Juxtacortical

Question 49

How many lesions are usually seen with enchondromas?

Answer 49

usually solitary

Question 50

What part of the bone is enchondroma usually associated with?

Answer 50

metaphysis of tubular bones of hands and feet

Question 51

What are they syndromes of multiple enchondromas?

Answer 51

1. Ollier disease: non hereditary syndrome of multiple formation enchondromas 2. Maffucci syndrome: multiple enchondromas form as well as soft tissue hemangiomas

Question 52

What do enchondromas cause?

Answer 52

pathologic fracture of phalanx

Question 53

What do enchondromas look like radiologically?

Answer 53

radiolucent nodules of hyaline cartilage scalloped endosteal surface

Question 54

What age group do you see giant cell tumors in?

Answer 54

20s to 40s

Question 55

Where do giant cell tumors occur

Answer 55

most commonly around the knee, can also be wrist

Question 56

Describe giant cell tumors

Answer 56

multinucleated osteoclast type giant cells. Benign but uncommon. Locally aggressive and can undergo cystic degeneration

Question 57

Does histology help in diagnosis of giant cell tumors? What should you watch out for?

Answer 57

yes- look for multinucleated giant cells. But you dont want to miss TB.

Question 58

Are you more likely to get malignant primary bone tumor or secondary?

Answer 58

secondary

Question 59

Half of primary malignant bone tumors are derived from what?

Answer 59

blood forming cells and connective tissue cells like, the commenest condition being MM

Question 60

Sites from which metastases occur to the bone are:

Answer 60

breast, prostate, lung, kidney, thyroid cancers

Question 61

____ is the most common primary bone tumor

Answer 61

osteogenic sarcoma

Question 62

Where is osteosarcoma commonly seen?

Answer 62

knee joing

Question 63

Where is chondrosarcoma seen?

Answer 63

trunk and limb girdles

Question 64

Where is ewings sarcoma usually seen?

Answer 64

diaphysis of bone

Question 65

75% of osteogenic sarcomas occur in what age group?

Answer 65

people under 20

Question 66

There is a peak of osteogenic sarcomas in the elderly that have what?

Answer 66

Paget's disease or irradiation

Question 67

What cancer associations can be made with osteogenic sarcoma?

Answer 67

Retinoblastoma, Rb, M2M2, 20% have lung metastases at presentation

Question 68

Symptoms of osteogenic sarcoma

Answer 68

Painful lesions that are progressively enlarging and cause fracture. Often these come to attention because of some other sporting injury.

Question 69

What is different about the location of osteogenic sarcomas in the elderly?

Answer 69

seen in bones of pelvis and skull

Question 70

radiological appearance of osteosarcoma (osteogenic sarcoma)

Answer 70

Lifted periosteum forms triangular shell of reactive bone; CODMAN TRIANGLE. Prominant bone formation that extends into soft tissue.

Question 71

Age group for chondrosarcoma

Answer 71

35-60

Question 72

____ is the 2nd most common primary malignant bone tumor.

Answer 72

Chondrosarcoma

Question 73

Chondrosarcoma may arise with preexisting ____.

Answer 73

enchondroma

Question 74

XRAY appearance of chondrosarcoma

Answer 74

can traverse joint spaces, appears lytic but isnt, scalloped endosteal surfaces

Question 75

Age group of Ewing's sarcoma

Answer 75

white 10-15

Question 76

85% of Ewing's sarcoma have what genetic signature?

Answer 76

PNETs: t(11:22)--> relate them to neuroectodermal tumors

Question 77

Survival rate of Ewing's sarcoma?

Answer 77

75% 5yr survival with surgery and chemo

Question 78

Where do Ewing's sarcomas occur?

Answer 78

diaphysis of long bone- arise in medulla

Question 79

What do ewings sarcomas look like on x-ray?

Answer 79

onion skin

Question 80

______ is a small round blue cell tumor

Answer 80

Ewings sarcoma

Question 81

What do Ewings sarcoma look like histologically?

Answer 81

Small round blue cells, glycogen rich, Homer-wright rossettes: tumor cells are arranged in a circle about a central fibrillary space- possible neural differentiation

Question 82

_____ are the most common tumors in bone

Answer 82

metastatic bone tumors

Question 83

in adults, over 75% of metastatic bone tumors arise from _____.

Answer 83

prostate, breast, lung, and kidney

Question 84

What metastatic bone tumors are common in children?

Answer 84

neuroblastoma, wilms tumor, osteosarcoma, ewing sarcoma, and rhabdomyosarcoma

Question 85

Describe metastatic bone tumors

Answer 85

usually multifocal, vertebrae, pelvis, ribs, skull, sternum, lytic, blastic, or mixed lytic and blastic

Question 86

_____ secrete substances that promote bone resorption

Answer 86

metastatic bone tumros

Question 87

What do metatstatic bone tumors from renal cancer look like

Answer 87

white neoplasms with red centers that bleed easily and can have hemosiderin

Question 88

Breast carcinoma metastases are mostly ____.

Answer 88

sclerotic