Chapter 144 - Malignant Bone Tumors

Question 1

osteosarcoma - presentation

Answer 1

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

Question 2

osteosarcoma - imaging

Answer 2

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

Question 3

osteosarcoma - path

Answer 3

gross: soft fleshy mass

low power histo: “frankly sarcomtous stroma” with tumor osteoid

osteosarcoma defined by presence of malignant osteoid

Question 4

osteosarcoma - treatment

Answer 4

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

Question 5

what is the most important prognostic indicator in osteosarcoma

Answer 5

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

Question 6

most common site of mets in osteosarcoma

Answer 6

1. lungs
2. bones

Question 7

chemotherapy drugs used for osteosarc

Answer 7

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

Question 8

paroseal osteosarcoma presentation

Answer 8

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

Question 9

parosteal osteosarcoma: imaging

Answer 9

dense, lobulated stuck on lesion
tumor is more dense in middle and least ossified peripherally

Question 10

parosteal osteosarcoma: path

Answer 10

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

Question 11

parosteal osteosarcoma: treatment

Answer 11

wide resection

Question 12

periosteal osteosarcoma

Answer 12

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

Question 13

telangiectatic oteosarcoma

Answer 13

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

Question 14

undifferentiated pleomorphic sarcoma of bone: presentation

Answer 14

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

Question 15

undifferentiated pleomorphic sarcoma of bone: imaging

Answer 15

lytic destructive lesion, variable periosteal reaction, cortical destruction, soft tissue mass

Question 16

undifferentiated pleomorphic sarcoma of bone: path

Answer 16

storiform, marked pleomorphism, lots of angry mitotic figures

Question 17

undifferentiated pleomorphic sarcoma of bone: treatment

Answer 17

neoadjuvant chemo
wide resection
postoperative chemo

survival slightly worse than traditional osteosarcoma with mets to lung and bones

Question 18

chondrosarcoma: presentation

Answer 18

adult patients: 40-70
slight male predominance

most common locations:
pelvis
proximal femur
scapula

Question 19

chondrosarcoma: treatment

Answer 19

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

Question 20

clear cell chondrosarcoma: presentation

Answer 20

epiphysis of long bones
most commonly: proximal femur, proximal humerus

Question 21

clear cell chondrosarcoma: imaging

Answer 21

lytic, round, well defined lesion, no periosteal reaction
+/- mineralization
MOST OFTEN CONFUSED FOR A BENIGN CHONDROBLASTOMA

Question 22

Clear cell chondrosarcoma: path

Answer 22

intermediate to high grade lesion with immature chondroytes

benign giant cells thruout tumor
EXTENSIVE clear cytoplasm with minimal matrix

Question 23

clear cell sarcoma: treatment

Answer 23

mets to lungs, get a CT chest (dont need abdomen/pelvis)

wide resection required

no role of chemo or rads

Question 24

ewing sarcoma: presentation

Answer 24

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

Question 25

ewing sarcoma: imaging

Answer 25

lytic
periosteal reaction in multiple layers: onion skinning or sunburst pattern
extensive soft tissue mass
need MRI to characterize soft tissue extent

Question 26

ewing sarcoma: path

Answer 26

gross: liquid consistency, mimicking pus

histo: sheets of small round blue cells with giant nuclei

Question 27

standard treatment of ewings

Answer 27

vincristine
doxorubicin/adriamycin
ifosfamide
etoposide
cytotoxan
actinomycin c

surgical wide resection for isolated extremity lesions

radiation for inoperabel tumors

Question 28

prognosis of ewings

Answer 28

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

Question 29

chordoma: presentation

Answer 29

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

Question 30

chordoma: imaging

Answer 30

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

Question 31

Chordoma: path

Answer 31

lobular and jelly like - tracks along nerve roots

hispt: physaliferous cell (contains vacuoles that appear bubly - cytoplasmic mucous)

KERATIN POSITIVE

Question 32

chordoma: tx

Answer 32

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

Question 33

adamantinoma

Answer 33

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

Question 34

multiple myeloma

Answer 34

age >40
african americans 2: 1 caucasians
males>females

Question 35

solitary plasmacytoma

Answer 35

treat with radiation alone