Volume 2

Classic osteosarcoma-----------------Case 108-9 & 451-490

Bone forming pseudotumors-----Case 491-498

Classic

Osteogenic

Sarcoma

Classic Osteogenic Sarcoma

Osteogenic sarcoma is the most common primary malignant

tumor of bone, making up 20 % of all primary malignancies,

with approximately 500-1000 new cases diagnosed each year in

the United States. The classic or most common form of osteo-

sarcoma is seen typically in children and young adults, with a

male preference. It occurs in the metaphyseal areas of fast growing

bones with the most common location being the distal femur,

second the proximal tibia, and third the proximal humerus.

50% of the lesions will be found around the knee joint. This tumor

is rare in in small bones such as the hand or the foot, or in vertebral

segments. Patients usually present with spontaneous symptoms

of pain in the area, followed several month later with a tumor

mass that is usually diagnosed by biopsy within six months after

onset of symptoms. The radiographic appearance of the lesion

is typically a permeative lytic lesion seen in the metaphyseal area

of a long bone with cortical breakthrough and periosteal elevation

creating a Codman’s reactive triangle, followed later by a sunburst

pattern of chaotic bone formation in the soft tissue outside the peri-

osteal sleeve. In a small percentage of cases, a so-called skip lesion

will appear as a separate nodule of tumor activity totally separate

from the primary lesion which, when found, suggests a very poor

prognosis for survival. Fifty percent of osteosarcomas are of the

osteoblastic type, but in a smaller percentage of cases, there will

be a prominence of cartilage or fibrous tissue that does not seem to

influence the prognosis for survival.

The staging process for this disease includes a MRI study of the

primary tumor that helps identify soft tissue invasion by the tumor

and defines the medullary extent of the tumor which helps the

operating surgeon determine the level of amputation or limb

salvage resection. A bone isotope scan is performed to rule out the

possibility of other bony foci in the skeletal system and a CT scan

of the chest is obtained to rule out the possibility of metastatic

disease to the lung. The final staging process includes a biopsy

of the primary site performed in such a way as to not contaminate

vital structures that might interfere with the potential for a limb

salvage resection at a later date.

Prior to 1970, the prognosis for survival with this disease was

only 20% even though early amputation was performed at a high

level. Pulmonary metastasis was the reason for a fatal outcome in

these early cases, however, with the advent of multi-drug chemo-

therapy the prognosis for survival has now increased to approx-

imately 60%. The drugs most commonly used for systemic control

of the disease include high dose methotrexate, adriamycin,

cysplatin, and ifosfamide. These drugs are administered through

a central venous line on a cyclic basis every three to four weeks

for approximately two months prior to a surgical removal of the

tumor. Chemotherapy is then continued for approximately four

months after surgical treatment.

At the present time, 90% of patients with osteosarcoma are

treated by limb salvage resection. The most common type of

reconstruction consists of a total joint replacement such as a

rotating hinge at the knee. A smaller group of patients are treated

with allograft reconstruction or combinations of the above.

Excisional arthrodesis was a popular technique many years ago

but now patients prefer a reconstruction that involves normal

joint motion. The prognosis for survival is influenced by the

degree of tumor necrosis produced by the preoperative chemo-

therapy protocol, so that at the time of surgical resection if there

is more than 90% necrosis of the tumor, the patient has a much

better prognosis for survival (approximately 85% at five years).

Pulmonary metastasis is still the major concern following treat-

ment for osteosarcoma and, if this does occur, aggressive surgical

resection of the lesions thru the chest wall is frequently performed.

There is a 30% survival rate at five years following this procedure.

As with other forms of cancer, recent molecular genetic studies

have revealed a high incidence of abnormality in the P-53

suppressor genes found in this tumor.

CLASSIC Case #108

16 yr male

classic OGS

femur

Bone scan

Sagittal T-1 MRI

tumor

Coronal T-2 MRI

Axial T-1 MRI

tumor

tumor

vessels

Axial T-2 MRI

tumor

CT scan with pulmonary mets to lung

Amputation

specimen

Macro section

Close up

Codman’s

triangle

tumor margin

Photomic

Higher power

High power

tumor

cells

Case #109

14 yr male

classic OGS

femur tumor

Coronal T-1 MRI

tumor

Coronal T-2 MRI

tumor

Distal femoral resection and reconstruction with

total knee replacement and Compress fixation

femur

measuring device

Widely resected tumor specimen

Reaming the

proximal tibia

Drill guide system

Placing 5 transverse pins

Traction bar protruding from femoral canal

Tightening the compression nut inside spindle

compression cap

compression nut

800 pounds of compressive fixation has been applied

intercalary

segment

spindle

Intercalary segment attached to spindle

Completion of rotating hinge arthroplasty

AP x-ray appearance

following surgery

anchor plug

spindle

Close up lateral

Stable osseointegration

5 years PO in another case

Case #655

16 year female

classic OGS

proximal femur

coronal T-2 MRI

Axial T-2 MRI

tumor

Widely resected specimen

Distal femoral stump being prepared for placement

of the spindle of the Compress reconstruction system

traction bar

Spindle fixed to femur with 800 lbs pressure

Proximal femoral replacement attached to spindle

spindle

Proximal end of modular system with bipolar hip

attachment point for abductor tendon

Hip located and ready for soft tissue attachments

Soft tissue reconstruction completed with two fixation screws

vastus lateralis

abductor tendon

fascia lata

screws

Resected specimen cut in path lab

tumor

Post op x-ray

5 yrs PO

Case #451

17 yr male

classic OGS

femur

Lateral view

Sagittal T-1 MRI

Proper biopsy site

Photomic

Resected specimen

biopsy

site

Specimen cut in

path lab showing

extensive tumor

necrosis

Surgical defect following wide resection

patella

Modular distal

resection system

with rotating hinged

knee

Rotating hinge

components horizontal

axial

vertical

axial

porous pads

Reconstruction

completed and

ready for closure

Radiographic

appearance

7 yrs later

stress

shielding

Case #452

13 year male with

Classic OGS distal femur

tumor

Codman’s

triangle

Sagittal T-1 MRI

tumor

tumor

vessels

Axial T-1 MRI

Photomic

Resected specimen

growth plate

Expandable

prosthesis with

telescoping sleeve

closed down

Telescoping

sleeve opened

Post op X-ray

Case #453

23 yr female

classic OGS

femur

tumor

Resected specimen

Photomic

Partially reconstructed

Completed reconstruction

Side view

Immediate post op

X-ray of cemented

stem prosthesis

13 yrs later with

total failure from

subsidence 2nd to

stress shielding

neck fracture

Surgical specimen

at time of total

femoral reconstruction

stress shielding

X-ray after total

femoral reconstruction

Case #454

17 yr male with classic OGS proximal femur

tumor

Lateral view

tumor

Bone scan

Coronal T-1 MRI

tumor

Axial T-1 MRI

tumor

vessels

Photomic

Modular proximal

femoral resection

system

Properly placed biopsy site over trochanter

incision

Wide resection

specimen

biopsy

site

femoral head

Cut specimen

in path lab

Surgical defect ready for reconstruction

acetabulum

Hyperemic synovium in acetabular notch

Suturing down

abductor tendon

to prosthesis

Final soft tissue

reconstruction

gluteus medius

vastus

lateralis

X-ray 7 yrs later

THA

Case #455

7 yr male classic OGS

distal femur

tumor

Bone scan

Sagittal T-1 MRI tumor

Coronal T-2 MRI

Axial T-1 MRI

vessels

tumor

Surgical incision for turn-up-plasty

Mobilizing prox tibia on vascular pedicle

vessels

tibia

femur

Resected distal femur

laying next to

inverted tibia

plate fixation

tibial plateau

Post op stump

appearance ready

for suction socket

prosthesis

Post op x-ray

prox tibial epiphysis

X-ray 18 mos later

tibial plateau

5 years later

Case #456

17 yr female

classic OGS with

pathologic fracture

and short plate fixation

10 mos post op wide

segmental resection

and double Compress

spacer reconstruction

Proximal Compress

device showing good

osseointegration

10 mos post op

Amputation specimen 10 mos post op

Excellent osseointegration at proximal end

anchor pins

Case #457

32 yr male

classic OGS

mid femur

Coronal T-2 MRI

Large extra

cortical mass

Axial T-2 MRI

fluid

tumor

Pathologic fracture after

6 weeks on chemotherapy

Coronal MRI

thru fracture site tumor

fracture

Gad contrast coronal MRI after 3 cycles of chemotherapy

necrotic

tumor rim

enhancement

Surgical specimen

following wide

resection

Specimen cut

in path lab

necrotic

tumor

fracture

Macro section

necrotic tumor

fracture

Photomic

Post op x-ray following

prosthetic reconstruction

Case #458

13 yr male

classic OGS

distal femur

tumor

Lateral view tumor

Bone scan

CT scan

tumor

T-1 axial MRI

tumor

tumor

edema

Coronal T-1 MRI

tumor

edema

Sagittal T-1 MRI

tumor

edema

Case #458.1

16 year old male with knee pain for 3 months

Cor T-1 T-2 Gad

Sag T-1 T-2 Gad

Axial T-1 T-2

Gad

Wide surgical resection and rotating hinge Compress recon

Case #458.2

8 year female with classic OGS distal femur

Cor T-1 MRI

Cor T-2 Cor Gad

Axial T-2

Axial Gad

Case #459

11 yr male

classic OGS

proximal tibia tumor

Lateral view

tumor

Coronal T-1 MRI tumor

Coronal T-2 MRI

tumor

Axial T-2 MRI

tumor

Photomic

15 year male with classic OGS proximal tibia

tumor

Case #461

Lateral view

tumor

Axial T-1 MRI

tumor

Macro section

tumor

Photomic

Case #461.1 AP & lat x-ray 3-05

17 year female dancer with prox. tibial pain for 3 mos with

early classic OGS looking like monototic fibrous dysplasia

6-05

CT scan 3 months later

Bone scan 7-05

Axial & sagittal T-1 MRI 6-05

Axial T-2 MRI

6-05

Axial T-1 FS Gad

6-05

AP & lat x-ray 5 mos later 11-05 & obvious OGS

Bone scan 11-05 biopsy proven OGS

and placed on preop chemotherapy

Coronal T-1 MRI 1-06 Sagittal T-1 MRI

Post chemo

Axial T-2 MRI 1-06 Sagittal T-2 MRI

following 2 mos of chemotherapy

X-ray following wide resection & Compress TKA

Case #462

14 year old female with

Classic OGS distal tibia tumor

AP view tumor

Macro section

tumor

Photomic

Case #463

14 year female

non-ossifying fibroma

tibia with no pain

Incidental finding

4 years later

and no change

14 yrs from 1st x-ray with sudden growth of tumor

Bone scan

Sagittal T-2 MRI tumor

Axial T-2 MRI

tumor

Photomic shows high grade classic OGS

Case #464

14 year female

classic OGS fibula

Another view

tumor

Case # 465

8 year male with classic OGS proximal fibula

Codman’s triangle

tumor

Case # 466

17 year male

classic OGS

proximal humerus

tumor

Coronal T-1 MRI

tumor

Axial T-2 MRI tumor

Widely resected

surgical specimen tumor

bulge

humeral

head

Specimen cut

in path lab

Photomic

Surgical reconsruction

with allograft and long

stem Neer prosthesis allograft

cement

Neer

Post op x-ray

Neer

allograft

Case #467

14 year female with

classic OGS proximal

humerus

Resected specimen

tumor

Cemented custom

prosthesis 5 years

post op

Case 468

16 year male with

classic OGS prox

humerus

Widely resected

surgical specimen

Cut specimen

in path lab

Photomic

Surgical defect

ready for

reconstruction

glenoid

Neer prosthesis

in position

Immediate post op

appearance

Case #468.1

18 year old male with

classic OGS proximal

humerus

tumor

Widely resected

specimen

Surgical defect

ready for

reconstruction

glenoid

Cemented Neer

prosthesis in

position cement

Appearance 9 mos later

with proximal migration

of prosthesis

mets

Case #468.2

14 year male

classic OGS

mid humerus tumor

Close up x-ray

after 1 mo of chemo

T-1 MRI after 2 cycles

of chemotherapy

T-2 MRI after 2 cycles

of chemotherapy

Axial PD MRI

tumor

Surgical specimen

from shoulder

disarticulation

Photomic

Case #468.3

15 year female with

Classic OGS proximal

Humerus with path fracture

Another view

fracture

Case #469 CT scan

27 year female with classic OGS 10th rib

2 years later develops 2nd OGS in R ilium

tumor

CT scan thru tumor

tumor

Another CT cut

tumor

Bone scan

Resected hemipelvis

tumor bulge

acetabulum

Surgical specimen

after 3 mins in

autoclave to kill

tumor ready for

reimplantation sciatic notch

acetabulum

Autoclaved pelvis reimplanted with total hip reconstruction

Post op x-ray appearance

X-ray 2 years later with fracture thru ilium

Case #470

18 year male with classic OGS pelvis

T-2 coronal MRI

tumor

Axial T-2 MRI

tumor

Entire hemipelvic resection specimen

Total hip reconstruction

prior to cementation

Cement construction

completed cement

constrained

total hip

Immediate post op x-ray

CD rod

Immediate post op

X-ray showing CD

rod reconstruction

X-ray 2.5 years later

Case #471

14 year male with classic OGS pelvis

tumor

CT scan

tumor

Axial T-2 MRI

tumor

Coronal T-2 MRI

tumor

spared

acetabulum

Rebar and cement reconstruction sparing hip

cement

X-ray and CT appearance

10 years later

X-ray appearance

Following THA

Case #472

26 year male with incidental fibrous cortical defect in ilium

12 years later with classic OGS in same area

Hemipelvic resection

including hip joint tumor

bulge

sciatic

notch

Reconstruction with

autoclaved hemipelvis

and cemented total hip

autoclaved

bone

THA

Completed

reconstruction

cement

X-ray appearance two years later

One year later the tumor recurred requiring the

removal of the hip reconstruction as we see in

this x-ray following which he died 1 yr later

Case #473

23 year male

classic OGS

lumbo-sacral spine tumor

Lateral X-ray

tumor

L-5

CT scan at L-5 - S-1 level

tumor

Photomic

Case #474

21 year male

classic OGS L-3

Bone scan

CT scan

tumor

L-3

Sagittal T-2 MRI

tumor

Photomic

Post op x-ray following

wide resection of L-3

and reconstruction with

anterior allograft and

pedicle screws and plates

allograft

Case #475

45 year female with classic OGS L-4

Sagittal T-1 MRI

tumor

Axial T-2 MRI

tumor

CT scan

tumor

Case #476

20 year male

classic OGS

first metatarsal

Lateral view

Photomic

Case #477

76 year female with classic OGS first metatarsal

Lateral x-ray

tumor

Case #478

17 year male

classic OGS

great toe

18 mos later

without treatment

Bone scan

Post op x-ray following

resection and cancellous

allograft reconstruction

Case #479

18 year female with classic OGS 4th metacarpal

Coronal gad contrast MRI

Axial gad contrast MRI

Another gad contrast cut

2 year post op x-ray with allograft reconstruction

Case #480

70 year male with soft tissue OGS foot

AP view

Photomic

Case #481

55 year male with classic OGS talus

tumor

Mortise view

tumor

Case #482

19 year male with classic OGS os calcis

Macro section

tumor

subtalar joint

Case #483

40 year female with classic OGS mandible

Cut surgical specimen following hemimandibulectomy

tumor

Case #484

75 year female

classic OGS

mandible

tumor

Case #485

36 year male with classic OGS lower rib

18 mos later and no treatment

enlarged

tumor

Bone scan

Case #486

25 year male with classic OGS rib

tumor

CT scan

Another CT cut

tumor

Photomic

Case #487

29 year female with classic OGS clavicle

tumor

Laminogram cut thru tumor

tumor

Immediate post op x-ray following resection

Case #488

21 year male with classic OGS patella

Patellar view of tumor

Case #489

19 year female

classic OGS

ulna

Case #490

38 year male

classic OGS

scapula

tumor

Bone Forming Pseudotumors

Stress fractures

Caffey’s disease

Brown tumor of hyperparathroidism

Hemophilia

Compartment syndrome [late]

Giant bone islands

Osteogenesis imperfecta

Case #491

14 year old female with

OGS pseudotumor tibia

(stress fracture)

Bone scan

Coronal T-1 MRI

Axial T-2 MRI

edema

Photomic of callus formation

Case #492

6 mo infant with pseudo OGS ulna which is Caffey’s disease

Photomic of ulnar biopsy

Transverse ulnar cut of amputation specimen

reactive

periostitis

cortex

X-ray showing hypertrophic changes in shoulder girdle

Mandibular hypertrophic changes typical of Caffey’s

Case 493

25 year female with pseudo OGS distal femur

In reality a brown tumor of hyperparathyroidism

Hemorrhagic giant cell response of brown tumor

Thickened osteoid seams of hyperparathyroidism

Case #494

12 year old male with

OGS pseudotumor distal

femur 2nd to pathologic

fracture in hemophilia

Lateral view

pseudotumor

Case #495

44 year male with old

crush injury to leg

25 yrs ago with

ossifying compartment

syndrome looking like

soft tissue OGS

Case #496

64 year female with pseudo OGS distal femur

in fact is a giant bone island

Lateral view

Bone scan

Coronal MRI with low signal lesion

Case #497

10 year female with

OGS pseudotumor from

osteogenesis imperfecta

large fluffy

callus

X-ray 2.5 years later

with healing fracture

Case #498

14 year male with OGS

pseudotumor second to

chronic stress fracture

proximal femur

Biopsy shows hypertrophic reactive bone and no OGS