Chapter 144 - Malignant Bone Tumors Flashcards
osteosarcoma - presentation
most common malignant bone tumor in children
slight predilection for boys
age 10-20 (and second peak >65)
increased incidence in p53 mutations, Rb mutations
intermittent pain, progressing to non-remitting nighttime and rest pain not relieved by meds
common location:
distal femur metaphysis
proximal tibia metaphysis
proximal humerus
pelvis
osteosarcoma - imaging
most tumors do not extend past the epiphyseal plate
cortical destruction and large soft tissue mass
classically originate in the medullary canal
codman’s triangle
mixed lytic and blastic appearance
osteosarcoma - path
gross: soft fleshy mass
low power histo: “frankly sarcomtous stroma” with tumor osteoid
osteosarcoma defined by presence of malignant osteoid
osteosarcoma - treatment
chemo - surgery - chemo
no real role for radiation
chemo used: doxorubicin, adriamycin, cisplatin, methotrexate, and ifosfamide
can use radiation in palliative inoperable primary tumors/mets
what is the most important prognostic indicator in osteosarcoma
tumor stage
percent of necrosis following chemo is related to overall survival (>90% necrosis -> good survival)
elevated LDH and alk phos, Veg-f = poor prognosis
most common site of mets in osteosarcoma
- lungs
- bones
chemotherapy drugs used for osteosarc
adriamycin/doxorubicin: blocks DNA synthesis, inhibits topoisomerase II
- cardiotoxicity
cisplatin: DNA disruption by covalent bonding
- hearing loss
- renal failure
- neuropathy
methotrexate: inhibits dihydrofolate reductase -> no dna synthesis
- allopecia
- mucositis
ifosfamide: dna-alkylating agent
- renal failure
- encephalopathy
paroseal osteosarcoma presentation
low grade surface osteosarcoma, dense bone
female 2:1
age 20-45
swelling of long duration (like a decade)
75% are on the posterior aspect of the distal femur metaphysis
next: proximal tibia, proximal humerus
parosteal osteosarcoma: imaging
dense, lobulated stuck on lesion
tumor is more dense in middle and least ossified peripherally
parosteal osteosarcoma: path
regular, ordered trabeculae
bland fibrous stroma with occasional atypical cells
***Dedifferentiated will be a biphasic histo - high grade sarcome juxtaposed next to low grade lesion
parosteal osteosarcoma: treatment
wide resection
periosteal osteosarcoma
extremely rare
kids 15-25
intermediate grade lesion
most commonly a tthe femoral or tibial diaphysis
imaging: sunburst periosteal elevation, no medullary involvement
path: extensive areas of chondroblastic matrix with osteoid
tx: chemo, wide resection, chemo
ten year survival is 77% with surgery, with ot with chemo
telangiectatic oteosarcoma
similar age and presentation of osteosarcoma
- 25% will present with pathologic fracture
imaging: purely lytic lesion on XR, MRI with fluid fluid levels and a large soft tissue component
histology: intervening septa of high grde sarcoma with atypical mitosis
tx: chemo-surgery-chemo like traditional osteosarc
undifferentiated pleomorphic sarcoma of bone: presentation
histiocytic differentiation and no osteoid
age 20-80 (most >40), slight male predominance
25% present as secondary tumor in setting of bone infarct, pagets, prior radiation
pain, swelling, limp
location: metaphysis of long bones - distal femur, proximal tibia, proximal humerus
undifferentiated pleomorphic sarcoma of bone: imaging
lytic destructive lesion, variable periosteal reaction, cortical destruction, soft tissue mass
undifferentiated pleomorphic sarcoma of bone: path
storiform, marked pleomorphism, lots of angry mitotic figures
undifferentiated pleomorphic sarcoma of bone: treatment
neoadjuvant chemo
wide resection
postoperative chemo
survival slightly worse than traditional osteosarcoma with mets to lung and bones
chondrosarcoma: presentation
adult patients: 40-70
slight male predominance
most common locations:
pelvis
proximal femur
scapula
chondrosarcoma: treatment
grade 1: intralesional curettage, or wide resection
pelvic lesions: need a clear margin
grde 2 or 3 get wide resection
lung mets get thoracotomy
no role for chemo in chondrosarc except de-diff
clear cell chondrosarcoma: presentation
epiphysis of long bones
most commonly: proximal femur, proximal humerus
clear cell chondrosarcoma: imaging
lytic, round, well defined lesion, no periosteal reaction
+/- mineralization
MOST OFTEN CONFUSED FOR A BENIGN CHONDROBLASTOMA
Clear cell chondrosarcoma: path
intermediate to high grade lesion with immature chondroytes
benign giant cells thruout tumor
EXTENSIVE clear cytoplasm with minimal matrix
clear cell sarcoma: treatment
mets to lungs, get a CT chest (dont need abdomen/pelvis)
wide resection required
no role of chemo or rads
ewing sarcoma: presentation
male to female 3:2
80% <20yo
uncommon in african american and chinese
classic 11;22 ews-fli translocation
frequently have fever and overlying erythema on presentation
elevated esr, ldh, ebc
most common location: pelvis -> diaphysis of long bones->scapula
these kids need a bone marrow biopsy on eval in addition to normal stagins tudies
ewing sarcoma: imaging
lytic
periosteal reaction in multiple layers: onion skinning or sunburst pattern
extensive soft tissue mass
need MRI to characterize soft tissue extent
ewing sarcoma: path
gross: liquid consistency, mimicking pus
histo: sheets of small round blue cells with giant nuclei
standard treatment of ewings
vincristine
doxorubicin/adriamycin
ifosfamide
etoposide
cytotoxan
actinomycin c
surgical wide resection for isolated extremity lesions
radiation for inoperabel tumors
prognosis of ewings
necrosis response to chemo is prognostic (>99% necrosis -> improved survival)
present with mets: <20% 5 year survival
mets to lung is better prognostic than non-pulm mets
chordoma: presentation
notochordal cells
male 3:1
>40 years
frequently misdiagnosed as OA, nerve impingement, or disk herniation
occur below S1 -> bowel bladder sx common
DO NOT DO A TRANSRECTAL BIOPSY - then youve contaminated the colon, rectal region, etc
chordoma: imaging
midline sacrum most common
usually cant see on plain xr - need CT 2/2 bowel gas
areas of calcification on CT
MRI:
- low T1
- High T2
Chordoma: path
lobular and jelly like - tracks along nerve roots
hispt: physaliferous cell (contains vacuoles that appear bubly - cytoplasmic mucous)
KERATIN POSITIVE
chordoma: tx
wide resection - like aggressive - get ready to sacrifice viscera, nerve roots, etc
+/- radiation
chemo has no role
mets late to lungs, need serial chest ct
adamantinoma
males 20-40
history of preceding trauma
anterior tibial cortex or whole tibia
soap bubble lesion of tibial diaphysis
nests of keratin positive epithelial cells in benign fibros stroma
tx = wide surgical resection, no chemo, no rads, often need intercalary allograft
multiple myeloma
age >40
african americans 2: 1 caucasians
males>females
solitary plasmacytoma
treat with radiation alone
