Volume 2

Classic osteosarcoma-----------------Case 108-9 & 451-490 Bone forming pseudotumors-----Case 491-498

Classic Classic Osteogenic Osteogenic SarcomaSarcoma

Classic Osteogenic Sarcoma Osteogenic sarcoma is the most common primary malignanttumor of bone, making up 20 % of all primary malignancies,with approximately 500-1000 new cases diagnosed each year inthe United States. The classic or most common form of osteo-sarcoma is seen typically in children and young adults, with amale preference. It occurs in the metaphyseal areas of fast growingbones 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 symptomsof 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 lesionis 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 sunburstpattern 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 separatefrom 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 tumorand 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 thepossibility of other bony foci in the skeletal system and a CT scanof the chest is obtained to rule out the possibility of metastatic

disease to the lung. The final staging process includes a biopsyof the primary site performed in such a way as to not contaminatevital structures that might interfere with the potential for a limbsalvage resection at a later date. Prior to 1970, the prognosis for survival with this disease wasonly 20% even though early amputation was performed at a high level. Pulmonary metastasis was the reason for a fatal outcome inthese 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 controlof the disease include high dose methotrexate, adriamycin, cysplatin, and ifosfamide. These drugs are administered througha central venous line on a cyclic basis every three to four weeksfor approximately two months prior to a surgical removal of the tumor. Chemotherapy is then continued for approximately fourmonths after surgical treatment. At the present time, 90% of patients with osteosarcoma are

treated by limb salvage resection. The most common type ofreconstruction consists of a total joint replacement such as a rotating hinge at the knee. A smaller group of patients are treatedwith 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 thereis more than 90% necrosis of the tumor, the patient has a muchbetter 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 studieshave revealed a high incidence of abnormality in the P-53 suppressor genes found in this tumor.

CLASSICCase #108

16 yr maleclassic OGSfemur

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

Amputationspecimen

Macro section

Close up

Codman’striangle

tumor margin

Photomic

Higher power

High power

tumorcells

Case #109

14 yr maleclassic OGSfemur 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

intercalarysegment

spindle

Intercalary segment attached to spindle

Completion of rotating hinge arthroplasty

AP x-ray appearancefollowing surgery

anchor plug

spindle

Close up lateral

Stable osseointegration5 years PO in another case

Case #655

16 year femaleclassic OGSproximal femur

coronal T-2 MRI

Axial T-2 MRI

tumor

Widely resected specimen

Distal femoral stump being prepared for placementof 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 tendonfascia lata

screws

Resected specimen cut in path lab

tumor

Post op x-ray

5 yrs PO

Case #451

17 yr maleclassic OGSfemur

Lateral view

Sagittal T-1 MRI

Proper biopsy site

Photomic

Resected specimenbiopsysite

Specimen cut inpath lab showingextensive tumornecrosis

Surgical defect following wide resection

patella

Modular distal resection systemwith rotating hingedknee

Rotating hingecomponents horizontal

axial

vertical axial

porous pads

Reconstructioncompleted and ready for closure

Radiographicappearance 7 yrs later

stress shielding

Case #452

13 year male withClassic OGS distal femur

tumor

Codman’striangle

Sagittal T-1 MRI

tumor

tumor

vessels

Axial T-1 MRI

Photomic

Resected specimen

growth plate

Expandable prosthesis with telescoping sleeveclosed down

Telescopingsleeve opened

Post op X-ray

Case #453

23 yr femaleclassic OGSfemur

tumor

Resected specimen

Photomic

Partially reconstructed

Completed reconstruction

Side view

Immediate post opX-ray of cementedstem prosthesis

13 yrs later with total failure fromsubsidence 2nd tostress shielding

neck fracture

Surgical specimenat time of totalfemoral reconstruction

stress shielding

X-ray after totalfemoral reconstruction

Case #454

17 yr male with classic OGS proximal femur

tumor

Lateral view

tumor

Bone scan

Coronal T-1 MRItumor

Axial T-1 MRI

tumor

vessels

Photomic

Modular proximalfemoral resectionsystem

Properly placed biopsy site over trochanter

incision

Wide resectionspecimen

biopsysite

femoral head

Cut specimenin path lab

Surgical defect ready for reconstruction

acetabulum

Hyperemic synovium in acetabular notch

Suturing downabductor tendonto prosthesis

Final soft tissuereconstruction

gluteus medius

vastus lateralis

X-ray 7 yrs later

THA

Case #455

7 yr male classic OGSdistal femur

tumor

Bone scan

Sagittal T-1 MRItumor

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 femurlaying next toinverted tibia

plate fixation

tibial plateau

Post op stumpappearance readyfor suction socketprosthesis

Post op x-ray

prox tibial epiphysis

X-ray 18 mos later

tibial plateau

5 years later

Case #456

17 yr femaleclassic OGS withpathologic fractureand short plate fixation

10 mos post op widesegmental resectionand double Compressspacer reconstruction

Proximal Compressdevice showing goodosseointegration10 mos post op

Amputation specimen 10 mos post op

Excellent osseointegration at proximal end

anchor pins

Case #457

32 yr maleclassic OGSmid femur

Coronal T-2 MRI

Large extracortical mass

Axial T-2 MRI

fluid

tumor

Pathologic fracture after6 weeks on chemotherapy

Coronal MRIthru fracture site

tumorfracture

Gad contrast coronal MRI after 3 cycles of chemotherapy

necrotictumor rim

enhancement

Surgical specimenfollowing wideresection

Specimen cut in path lab

necrotictumor

fracture

Macro section

necrotictumor

fracture

Photomic

Post op x-ray followingprosthetic reconstruction

Case #458

13 yr maleclassic OGSdistal femur

tumor

Lateral viewtumor

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 OGSproximal tibia tumor

Lateral view

tumor

Coronal T-1 MRItumor

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 #461.1AP & lat x-ray 3-05

17 year female dancer with prox. tibial pain for 3 mos withearly 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 withClassic OGS distal tibia tumor

AP view tumor

Macro section

tumor

Photomic

Case #463

14 year femalenon-ossifying fibromatibia with no pain

Incidental finding

4 years laterand 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 femaleclassic OGS fibula

Another view

tumor

Case # 465

8 year male with classic OGS proximal fibula

Codman’s triangle

tumor

Case # 466

17 year maleclassic OGSproximal humerus

tumor

Coronal T-1 MRI

tumor

Axial T-2 MRItumor

Widely resectedsurgical specimen

tumorbulge

humeralhead

Specimen cut in path lab

Photomic

Surgical reconsructionwith allograft and longstem Neer prosthesis allograft

cement

Neer

Post op x-ray

Neer

allograft

Case #467

14 year female with classic OGS proximal humerus

Resected specimentumor

Cemented customprosthesis 5 years post op

Case 468

16 year male withclassic OGS proxhumerus

Widely resected surgical specimen

Cut specimen in path lab

Photomic

Surgical defectready forreconstruction

glenoid

Neer prosthesisin position

Immediate post opappearance

Case #468.1

18 year old male withclassic OGS proximalhumerus

tumor

Widely resectedspecimen

Surgical defectready forreconstruction

glenoid

Cemented Neerprosthesis inposition

cement

Appearance 9 mos laterwith proximal migrationof prosthesis

mets

Case #468.2

14 year maleclassic OGSmid humerus

tumor

Close up x-rayafter 1 mo of chemo

T-1 MRI after 2 cyclesof chemotherapy

T-2 MRI after 2 cyclesof chemotherapy

Axial PD MRI

tumor

Surgical specimenfrom shoulderdisarticulation

Photomic

Case #468.3

15 year female with Classic OGS proximalHumerus 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 specimenafter 3 mins inautoclave to killtumor ready forreimplantation

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 reconstructionprior to cementation

Cement constructioncompleted

cement

constrainedtotal hip

Immediate post op x-ray

CD rod

Immediate post opX-ray showing CDrod 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

sparedacetabulum

Rebar and cement reconstruction sparing hip

cement

X-ray and CT appearance 10 years later

X-ray appearanceFollowing THA

Case #472

26 year male with incidental fibrous cortical defect in ilium

12 years later with classic OGS in same area

Hemipelvic resectionincluding hip joint

tumorbulge

sciaticnotch

Reconstruction withautoclaved hemipelvisand cemented total hip

autoclavedbone

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 maleclassic OGSlumbo-sacral spine

tumor

Lateral X-ray

tumor

L-5

CT scan at L-5 - S-1 level

tumor

Photomic

Case #474

21 year maleclassic OGS L-3

Bone scan

CT scan

tumor

L-3

Sagittal T-2 MRI

tumor

Photomic

Post op x-ray followingwide resection of L-3and reconstruction withanterior allograft andpedicle 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 OGSfirst metatarsal

Lateral view

Photomic

Case #477

76 year female with classic OGS first metatarsal

Lateral x-ray

tumor

Case #478

17 year male classic OGSgreat toe

18 mos laterwithout treatment

Bone scan

Post op x-ray followingresection and cancellousallograft 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 femaleclassic OGS mandible

tumor

Case #485

36 year male with classic OGS lower rib

18 mos later and no treatment

enlargedtumor

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 femaleclassic OGSulna

Case #490

38 year maleclassic OGSscapula

tumor

Bone Forming Bone Forming PseudotumorsPseudotumors

Stress fracturesCaffey’s diseaseBrown tumor of hyperparathroidismHemophiliaCompartment syndrome [late]Giant bone islandsOsteogenesis imperfecta

Case #491

14 year old female withOGS 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

reactiveperiostitis

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 withOGS pseudotumor distalfemur 2nd to pathologicfracture in hemophilia

Lateral view

pseudotumor

Case #495

44 year male with oldcrush injury to leg 25 yrs ago with ossifying compartmentsyndrome looking likesoft 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 withOGS pseudotumor fromosteogenesis imperfecta

large fluffycallus

X-ray 2.5 years laterwith healing fracture

Case #498

14 year male with OGSpseudotumor second tochronic stress fractureproximal femur

Biopsy shows hypertrophic reactive bone and no OGS