090814 bone tumors Flashcards
incidence of bone tumors
relatively rare kind of tumors
malignant bone tumors are even rarer
clinical presentation of bone tumor
usually nonspecific
may have: pain mass pathologic fracture asymptomatic
factors to consider in diagnosis of bone tumor
age
sex
skeletal localization (which bone, which area of bone?)
radiographic appearance
juxtacortical means
by the periosteum
what kinds of bone tumors are associated more with childhood and adolescence?
osteosarcoma
Ewing’s sarcoma
what kinds of bone tumors are associated more with young adults
giant cell tumor
what kinds of bone tumors are associated more with elderly?
chondrosarcoma
locations of bone lesions?
see slide 6
on radiology, implications of a sclerotic margin
usually indicates a benign and slowly growing neoplasm
if margin is illdefined, then is typically a maligant and fast growing neoplasm
bone vs cartilage matrix-forming tumor on radiography
bone matrix forming: is solid and ivory like
cartilage matrix forming: rings and arcs
ex of benign bone forming tumors
osteoid osteoma
osteoblastoma
diff btwn osteoid osteoma and osteoblastoma
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
osteoid osteoma looks like histologically
immature bone lined by osteoblasts
osteoblastoma looks like histologically
similar to osteoid osteoma
osteosarcoma
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
where does osteosarcoma occur
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
what elements can cause osteosarcoma
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
osteosarcoma on imaging
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)
pathology of osteosarcoma under microscope
infiltrative tumor, extends into soft tissue
malignant cells making osteoid
tripolar mitoses
larger cells with prominent nucleoliand folding of nuclei
prognosis for osteosarcoma
after chemotherapy came along, much better prognosis
use chemo
resection following chemo–this leads to 90% necrosis and near 90% survival rate
osteochondroma
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
enchondroma-define and at what sites
benign hyaline cartilage lesion
intramedullary chondroma
small bones of hands and feet, appendicular skeleton
periosteal chondroma
juxtacortical chondroma
xr of enchondroma shows
lytic, lobulated, cortical thinning
what are types of multiple chondromatosis?
ollier’s disease
Maffucci’s syndrome
multiple chondromatosis are characterized by frequent point mutations in IDH1 or IDH2
ollier’s disease vs maffucci’s syndrome
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
chondrosarcoma
malignant tumor in which neoplastic cells make purely cartilaginous matrix
second most common bone sarcoma
mainly older adults
where do chondrosarcomas occur
central skeleton-pelvis and ribs, humerus, femur
metaphysis and diaphysis; medullary
imaging of chondrosarcomas
medullary location
often have calcifications, which are lost in grade 3
cortical erosion
occasional soft tissue extension
popcorn like, white areas are calcifications
how to tell diff on pathology btwn enchondromas and chondrosarcomas?
chondrosarcomas usually are more cellular and nuclei are more pleomorphic
binucleation and myxoid change of matrix in malignant
non-ossifying fibroma
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
pathologic fracture
fracture due to thinning of bone
fibrous dysplasia
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
McCune Albright syndrome
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)
fibrous dysplasia radiography
expansile
circuscribed
thinned cortex
ground glass appearance (looks flat, lacks texture)
pathology of fibrous dysplasia
haphazard, randomly oriented woven bone trabeculae (Chinese characters) that’s surrounded by fibroblastic stroma
no significant osteoblastic rimming
treatment of fibrous dysplasia
conservative except for polyostotic form
Ewing sarcoma and primary neuroectodermal tumor (PNET)
common malignant bone tumor in childhood
males more than females
painful, often enlarging mass
diaphysis of long bones, ribs, pelvis
XR of Ewing sarcoma/PNET
destructive moth-eaten and permeative medullary lesion with large soft tissue mass
onion skin pattern of periosteal reaction in response to fast growth
pathology of Ewing sarcoma/PNET
sheets of primitive small, round, blue cells with neural phenotype
membranous CD 99 on immunostain
abundant glycogen
commonly see hemorrhage and necrosis
what genetic component is commonly seen in Ewing Sarcoma/PNET
t (11,22) –so EWS gene on 22q is fused with transcripton factor on 11q
giant cell tumor of bone
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
pathology of giant cell tumor
multinucleated giant cell–osteoclast like giant cells but much more nuclei
metatstaic bone tumor
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)
