090814 bone tumors

Question 1

incidence of bone tumors

Answer 1

relatively rare kind of tumors

malignant bone tumors are even rarer

Question 2

clinical presentation of bone tumor

Answer 2

usually nonspecific

may have:
pain
mass
pathologic fracture
asymptomatic

Question 3

factors to consider in diagnosis of bone tumor

Answer 3

age
sex
skeletal localization (which bone, which area of bone?)
radiographic appearance

Question 4

juxtacortical means

Answer 4

by the periosteum

Question 5

what kinds of bone tumors are associated more with childhood and adolescence?

Answer 5

osteosarcoma

Ewing’s sarcoma

Question 6

what kinds of bone tumors are associated more with young adults

Answer 6

giant cell tumor

Question 7

what kinds of bone tumors are associated more with elderly?

Answer 7

chondrosarcoma

Question 8

locations of bone lesions?

Answer 8

see slide 6

Question 9

on radiology, implications of a sclerotic margin

Answer 9

usually indicates a benign and slowly growing neoplasm

if margin is illdefined, then is typically a maligant and fast growing neoplasm

Question 10

bone vs cartilage matrix-forming tumor on radiography

Answer 10

bone matrix forming: is solid and ivory like

cartilage matrix forming: rings and arcs

Question 11

ex of benign bone forming tumors

Answer 11

osteoid osteoma

osteoblastoma

Question 12

diff btwn osteoid osteoma and osteoblastoma

Answer 12

osteoid osteoma:
long bones, femur, tibia
less than 2 cm
night pain
responds to aspirin
radiolucent lesion within sclerotic cortex

osteoblastoma:
vertebrae or long bone metaphysis
greater than 2 cm
pain
not responsive to aspirin
expansile radio-lucency

Question 13

osteoid osteoma looks like histologically

Answer 13

immature bone lined by osteoblasts

Question 14

osteoblastoma looks like histologically

Answer 14

similar to osteoid osteoma

Question 15

osteosarcoma

Answer 15

malignant mesenchymal tumor in which cells make osteoid or bone

most common sarcoma of bone

males more than females

60% is at age 10-20
also incidence peaks at 55-80

Question 16

where does osteosarcoma occur

Answer 16

metaphysis of long bones (femur, tibia, humerus), flat bones, spine (older pts)

could by polyostotic, meaning at multiple sites (but not common)

spreads commonly heamtogenously to lungs

Question 17

what elements can cause osteosarcoma

Answer 17

mutant allele of RB gene
mutation of p53 suppressor gene (Li Fraumeni-bone and soft tissue sarcomas, breast cancer, etc.)
overexpression of MDM2, INK4 and p16

sites of bone growth or disease (Paget’s disease)
prior irradiation

Question 18

osteosarcoma on imaging

Answer 18

poorly delineated
bone destruction
cortical disruption (won’t see nice outlines of bones)
bone matrix
soft tissue extension (can grow right through ththe bone and extend to soft tissue)

Codman’s sign (radiographic sign of rapidly growing mass and periosteum growing around it-outgrowth of periosteum extending outside the cortex)

Question 19

pathology of osteosarcoma under microscope

Answer 19

infiltrative tumor, extends into soft tissue
malignant cells making osteoid

tripolar mitoses
larger cells with prominent nucleoliand folding of nuclei

Question 20

prognosis for osteosarcoma

Answer 20

after chemotherapy came along, much better prognosis

use chemo
resection following chemo–this leads to 90% necrosis and near 90% survival rate

Question 21

osteochondroma

Answer 21

cartilaginous benign tumor of bone

most common benign tumor of bone

occurs in metaphysis of long bones

spurs that grow out off of the bone–get cartilage cap and bone underneath and then marrow underneath bone

Question 22

enchondroma-define and at what sites

Answer 22

benign hyaline cartilage lesion
intramedullary chondroma

small bones of hands and feet, appendicular skeleton

Question 23

periosteal chondroma

Answer 23

juxtacortical chondroma

Question 24

xr of enchondroma shows

Answer 24

lytic, lobulated, cortical thinning

Question 25

what are types of multiple chondromatosis?

Answer 25

ollier’s disease
Maffucci’s syndrome

multiple chondromatosis are characterized by frequent point mutations in IDH1 or IDH2

Question 26

ollier’s disease vs maffucci’s syndrome

Answer 26

both are multiple endchondromatas, but Maffucci’s syndrome includes angiomata

Maffucci’s syndrome has severe skeletal malfomration, whereas Ollier’s can have or not have this

Ollier’s disease tends to have regional distribution

Maffucci’s syndrome tends to have higher incidence of maligant transformation

Question 27

chondrosarcoma

Answer 27

malignant tumor in which neoplastic cells make purely cartilaginous matrix

second most common bone sarcoma

mainly older adults

Question 28

where do chondrosarcomas occur

Answer 28

central skeleton-pelvis and ribs, humerus, femur

metaphysis and diaphysis; medullary

Question 29

imaging of chondrosarcomas

Answer 29

medullary location
often have calcifications, which are lost in grade 3
cortical erosion
occasional soft tissue extension

popcorn like, white areas are calcifications

Question 30

how to tell diff on pathology btwn enchondromas and chondrosarcomas?

Answer 30

chondrosarcomas usually are more cellular and nuclei are more pleomorphic

binucleation and myxoid change of matrix in malignant

Question 31

non-ossifying fibroma

Answer 31

common developmental cortical defect-thinning
most comon space occupying lesion of bone (1 in 4)

tibia, femur (metaphysis) in first to third decades of life

eccentric, lytic, peripheral sclerosis

fibroblasts have storiform (starry night) pattern

Question 32

pathologic fracture

Answer 32

fracture due to thinning of bone

Question 33

fibrous dysplasia

Answer 33

benign tumor
all components of bone are there but they don’t differentiate–so is a developmental arrest

usually monostotic (seen in adolescents)

polyostotic occurs more often in infancy and childhood

Question 34

McCune Albright syndrome

Answer 34

polyostotic fibrous dysplasia with endocrinopathies and cafe au lait spots

rare form, females more than males

results in sexual precocity, acromegaly, Cushing syndrome

mutations of GNAS (GTP binding protein)

Question 35

fibrous dysplasia radiography

Answer 35

expansile
circuscribed
thinned cortex
ground glass appearance (looks flat, lacks texture)

Question 36

pathology of fibrous dysplasia

Answer 36

haphazard, randomly oriented woven bone trabeculae (Chinese characters) that’s surrounded by fibroblastic stroma

no significant osteoblastic rimming

Question 37

treatment of fibrous dysplasia

Answer 37

conservative except for polyostotic form

Question 38

Ewing sarcoma and primary neuroectodermal tumor (PNET)

Answer 38

common malignant bone tumor in childhood

males more than females

painful, often enlarging mass

diaphysis of long bones, ribs, pelvis

Question 39

XR of Ewing sarcoma/PNET

Answer 39

destructive moth-eaten and permeative medullary lesion with large soft tissue mass

onion skin pattern of periosteal reaction in response to fast growth

Question 40

pathology of Ewing sarcoma/PNET

Answer 40

sheets of primitive small, round, blue cells with neural phenotype

membranous CD 99 on immunostain

abundant glycogen

commonly see hemorrhage and necrosis

Question 41

what genetic component is commonly seen in Ewing Sarcoma/PNET

Answer 41

t (11,22) –so EWS gene on 22q is fused with transcripton factor on 11q

Question 42

giant cell tumor of bone

Answer 42

most benign, locally aggressive (may thin cortex and extend to soft tissue)

in young adults and skeletally matured older adolescents

in females more than males

epiphyseal location

can see hemokorahge with necrosis

Question 43

pathology of giant cell tumor

Answer 43

multinucleated giant cell–osteoclast like giant cells but much more nuclei

Question 44

metatstaic bone tumor

Answer 44

most common malignant bone tumor (moreso than primary)

mostly multiple tumors

solitary lesions may mimic primary bone tumor and be discovered before its source

70% in axial skeleton

commonly from breast, lung, thyroid, prostate, and kidney (BLT KP)