Transcript

Volume 5

Malignant Chondroid Tumors

Primary central chondrosarcoma----------Case 125 & 652-678

Secondary peripheral chondrosarcoma---Case 126 & 679-686

Dedifferentiated chondrosarcoma---------Case 127 & 687-689

Clear cell chondrosarcoma-----------------Case 128 & 690

Mesenchymal chondrosarcoma------------Case 129 & 691

Cartilaginous pseudotumors---------------Case 692-699

Chondrosacoma

Primary Central

Chondrosarcoma

Primary Central Chondrosarcoma The primary or central conventional chondosarcoma is a low

grade but malignant cartilagenous tumor found typically in adults

between the ages of 30 and 60 years. The tumor arises from the

medullary canal of a large bone such as the pelvis, femur, tibia or

proximal humerus. Because the tumor is slow growing, there is

little symptomatology and the tumor frequently becomes quite

sizable before a physician is consulted. Primary chondrosarcoma

is extremely rare in small bones of the hand or foot. The meta-

physeal portion of a long bone is the most common location

although diaphyseal locations are not unusual. 85% of central

chondrosarcomas are low grade lesions which on radiographic

examination demonstrate matrix calcification similar to that seen

in benign enhondromas, whereas the high grade chondrosarcomas,

which are rare, are frequently noncalcified and take on the

permeative appearance similar to other high grade sarcomas such

as fibrosarcoma and Ewing’s sarcoma. Histologically, the low

grade central chondrosarcoma has a fairly well differentiated

chondroid matrix like that of an enchondroma but shows evidence

of permeative invasion into the adjacent cortical and cancellous

structures. There is rarely any mitotic activity in the low grade

lesions. They have larger nuclear patterns with a higher degree

of atypicism compared to benign enchondromas.

These low grade tumors have a good prognosis in terms of a

low metastatic incidence to the lung but they must be treated

aggressively with a wide resection in order to prevent local

recurrence. One cannot rely on adjuvant therapy such as radiation

or systemic chemotherapy because these low grade lesions are

notoriously resistant to adjuvant therapy.

CLASSIC Case #125

50 year female with chondrosarcoma prox humerus

Axial T-1 MRI

Axial T-2 MRI

Proximal humeral

resection tumor bulge

humeral head

Macro section

Close up macro section

Photomic

Surgical defect

following wide

resection

glenoid

Proximal humeral

allograft ready for

implantation

rotator cuff

Allograft placed

over long stem

Neer prosthesis

pectoralis Neer

Alloprosthetic

reconstruction

completed

rotator

cuff

pectoralis

Post op X-ray

Case #652

74 female

chondrosacoma

proximal humerus

Bone scan

Coronal T-1 MRI

Coronal T-2 MRI tumor

Wide resection

proximal humerus

glenoid

Resected specimen cut in path lab

cortical erosion

Photomic

Suturing down the

allograft rotator cuff

as part of the alloprosthetic

reconstruction

Post op x-ray with

cemented Neer

alloprosthesis

Case #653

19 year male

chondrosarcoma

proximal humerus

Coronal proton density MRI

tumor

Resected specimen

cut in path lab

tumor

Macro section

Photomic

Case #654

28 year female with chondrosarcoma prox humerus

Coronal T-1 MRI

Axial proton density MRI

Axial T-2 MRI

tumor

Case #656

72 year male

chondrosarcoma

femur

Bone scan

Widely resected

distal femur specimen

cut in path lab

Close up showing

cortical break thru

Photomic

Case #657

83 year male

chondrosarcoma femur

Case #658

82 year female

chondrosarcoma

proximal femur

X-ray resected

proximal femoral

tumor with path

fractures

Macro section

Close up macro

Photomic

Case #659

52 year male

chondrosarcoma

mid femur

Another view

Bone scan

Coronal T-1 MRI

Macro section from

intercalary resection

tumor

Photomic showing bony permeation

tumor

bone

Photomic

Higher power

Post op X-ray

Case #660

54 year female with chondrosarcoma distal femur

Resected distal femur including entire knee joint

patella

Distal femur cut in path lab

tumor

Macro section

tumor

Photomic

tumor

tumor

articular cartilage

Close up photomic showing bone permeation by tumor

tumor bone

Post op x-ray with

excisional arthrodesis

I.M.

nail

Case #660.1

33 year male with painless mass in popliteal space for 1 yr

and restricted flexion of knee for 3 yrs

Sag T-1 T-2

Cor T-2 Axial T-2

Surgical specimen Compress rotating hinge

PO x-rays

Case #661

58 year male

chondrosarcoma

proximal tibia tumor

Lateral view

Resected specimen cut in path lab

Photomic

Proximal tibial allograft

placed over long stem

total knee replacement

with wires for patellar

tendon attachment

spherocentric knee

Patellar tendon

sutured to proximal

tibial allograft

Completion of

retinacular closure

patella

Post op x-ray

allograft

9 years later

Case #662

43 year male with chondrosarcoma proximal tibia

Coronal T-2 MRI

tumor

Sagittal Gad C MRI

tumor

Axial T-1 MRI tumor

Proximal tibial resection with tumor breakout posterior

Proximal tibial resection prosthesis with Compress System

spindle

anchor plug drill

Resected proximal tibia next to prosthesis

Drilling holes for

anchor plug pins

in tibia

guide

Placement of anchor plug and traction bar

Milling the proximal tibial stump

mill

Milling process

completed ready

for spindle placement

over traction bar

Spindle secured with 600 lbs of spring pressure

spindle

Femoral component of rotating hinge cemented in place

Components assembled ready for patellar ligament attachment

Spiked washers secure patellar ligament

Soft tissue reconstruction completed ready for closure

Post op x-ray

Case #663

63 year female

chondrosarcoma

pelvis

Internal hemipelvectomy resection specimen

acetabulum

sciatic

notch

Resected specimen after autoclaving

sciatic notch

acetabulum

Autoclaved specimen

replaced with routine

cemented total hip

Immediate post op

X-ray showing rebar

and cement fixation

upper resection line

15 years later

Case #664

54 year male with chondrosarcoma mid pelvis

CT scan

Bone scan

Type II internal hemipelvectomy resection

Type II resection

specimen tumor

femoral head

Photomic

Autoclaved specimen

Autoclaved specimen

reimplanted with

cemented total hip &

recon plates

Nine years later

Case #665

47 year female with chondrosarcoma mid pelvis

CT scan

tumor

Coronal T-1 MRI

tumor

Coronal T-2 MRI

tumor

Photomic

X-ray 1 year post op internal hemipelvectomy & THA

4 years post op

8 yrs PO with slight lateral shift of cup

Case #666

45 year female with chondrosarcoma mid pelvis

Coronal T-2 MRI

Cutting ilium with Gigli saw

ilium

Placement of 6.5 screws in ilium, ischium & ant ramus

ischium

ant ramus

Reconstruction of pelvic ring with recon plates

iliac screws

Placement of constrained cup prior to cementing

Cup cemented

Resected ilium & socket lying next to reconstruction

Hip relocated ready for greater troch attachment

Skin closure including biopsy site

Post op x-ray

Post op x-ray

Case #667

50 year male

chondrosarcoma

anterior acetabulum

Bone scan

Axial T-1 MRI

Type II & III resection and rebar and cement total hip

Case #667.1

78 yr male with primary chondrosarcoma pelvis

Axial T-1 MRI

Axial T-2 MRI

Axial Gad MRI showing rim enhancement

Coronal T-1 MRI

Coronal T-2 MRI

Coronal Gad MRI

Case #667.2

56 yr old female with prior excision of pelvic tumor 4 yrs ago

Recurrent chondrosarcoma

Cor T-1 T-2 Gad

Current MRI

Axial T-1 T-2

Gad

Sag gad Surgical specimen

Case #668

43 year male with chondrosarcoma body of scapula

7 years later

CT scan

Another CT cut

tumor

Coronal T-2 MRI

tumor

Axial T-2 MRI

tumor

Total scapular prosthesis

Cementing humeral component

Scapular component positioned in muscle cuff

Closure of muscle cuff over scapular component

Resected scapula and humeral head

tumor

bulge

Post op x-ray

Case #669

47 year male with chondrosarcoma scapular body

tumor

Another CT cut

tumor

Axial proton density MRI

tumor

Axial proton density MRI

tumor

Axial T-2 MRI

tumor

Coronal proton density MRI

tumor

Case #670

52 year male with chondrosarcoma elbow

Cut specimen in path lab

Photomic

Case #670.1

76 year female with slow growing chondrosarcoma elbow

Axial

T-1

T-2 T-2

Sag T-1 Sag STIR

Cor T-1 Cor STIR

Surgical debulking

Case #671

26 year male

chondrosarcoma

radius

Coronal T-1 MRI

Photomic

Case #672

36 year male with chondrosarcoma distal radius

CT scan

Case #673

56 year male

enchondroma

2nd metacarpal

3.5 years later with

chondrosarcoma

Photomic

Case #674

77 year female with chondrosarcoma os calcis

Os calcis view

T-1 MRI

T-2 MRI

tumor

Sagittal T-2 MRI

tumor

Photomic

Case #674.1

55 year male with slight pain and swelling about ankle for 2 years

Bone scan

CT Scan

Sag PD T-2

Gad

Axial PD T-2

Gad

Case #675

72 year male with chondrosarcoma chest wall

tumor

Lateral view

tumor

CT scan

Another CT cut

Photomic

Case #676

52 year male with chondrosarcoma rib

Macro section of resected specimen

tumor

rib

Photomic

Case #676.1

38 year male

chondrosarcoma

L-1 with block on

myelogram

CT scan

Photomic

Case #676.2

74 yr male with low grade chondrosarcoma LD spine 2 yrs

CT scan

Sag T-1 Sag T-2 Sag Gad

Axial T-1 Axial T-2 Axial Gad

Case #676.3

48 year old male with low back pain for 1 year

Chondrosarcoma L-3

Bone scan

Axial T-1 T-2

Sag T-2

Case #677

46 year male with chondrosarcoma mandible

Photomic

Case #678

39 year male

chondrosarcoma

mandible

Secondary

Peripheral

Chondrosarcoma

Secondary Peripheral Chondrosarcoma

The vast majority of secondary peripheral chondrosarcomas arise

from a prexisting osteochondroma and do not occur before puberty.

These lesions tend to be slow growing with minimal to mild

symptoms. The most common site is the pelvis, followed by the

proximal femur, proximal humerus, and ribs. Plain radiographs show

a large calcifying mass on the surface of bone that measure over

5 cm in girth. When one sees an osteochondroma with a cartilagenous

cap over 3 cm in thichness, there is a strong likelihood for a

secondary chondrosarcoma. The overall prognosis for the secondary

peripheral chondrosarcoma is much better than that for the primary

central chondrosarcoma and usually requires only a simple wide

resection with little chance for local recurrence.

CLASSIC Case #126

56 year male with 2ndary peripheral chondrosarcoma ilium

tumor exostosis

Coronal T-2 MRI

tumor

Resected specimen cut in path lab

ilium

Macro section

ilium

Low power photomic

Higher power with ditto forms

Case #679

60 year female with chondrosarcoma pubic area

Gross resection specimen

tumor

Gross specimen cut in path lab

pubic bone

tumor

Macro section

pubic bone

tumor

Close up macro section

pubic bone

Photomic

Post op x-ray

Case #680

31 year male

chondrosarcoma

ilium in multiple

exostosis patient

tumor

exostosis

Axial proton density MRI

tumor

exostosis

Axial T-2 MRI

tumor

tumor

Axial T-2 MRI

Photomic

X-ray of knees with multi exostoses

Case #681

18 year female

chondrosarcoma

pelvis and multi

hereditary exostoses

Oblique view

CT scan

Bone scan

tumor

Resected specimen

tumor

Macro section

Photomic

Multi exostoses knee

Lateral view

Several years after type I resection

Case #682

38 year female with osteochondroma C-spine

CT scan

Surgical photo at at same time

Surgical specimen cut in path lab

cap

Recurrence 3 years

later

Recurrence CT scan

Coronal T-1 MRI

of recurrence and

chondrosarcoma

tumor

Chondrosarcoma photomic

Case #683

42 year female with 2ndary chondrosarcoma ilium

CT scan

Coronal T-1 MRI

tumor

Axial T-1 MRI

tumor

Axial T-2 MRI

tumor

Case #684

33 year male

2ndary chondrosarcoma

os calcis

Lateral view

CT scan

Soft tissue CT scan

exostosis

chondrosarc

Coronal proton density MRI

tumor

Coronal T-2 MRI

tumor

Surgical specimen cut in path lab

Photomic

Case #685

30 year male with multi exostoses & chondrosarcoma chest wall

Case #686

42 year male with 2ndary chondrosarcoma

Macro section

bone stock

chondrosarc

Case #686A

42 year male with peripheral chondrosarcoma ulna

Dedifferentiated

Chondrosarcoma

Dedifferentiated Chondrosarcoma

Of all the chondrosarcoma variants, by far the most malignant and

potentially fatal is the dedifferentiated chondrosarcoma that

accounts for approximately 5-10% of all chondrosarcomas. It

most likely arises as a result of a second mutation within a pre-

existing low to intermediated grade chondrosarcoma, resulting in

the formation of a malignant fibrous histiocytoma or osteo-

sarcoma immediately adjacent to the lower grade chondrosarcoma

Histologically, the low and high grade portions of this tumor are

geographically separated by sharp margins. These tumors usually

occur in patients between the ages of 50 and 70 years in the

same areas where primary centeral chondrosarcomas are found:

the pelvis, femur and proximal humerus. The characteristic

radiographic appearance is that of a flocculated calcific lytic

lesion arising from the central area of bone with an adjacent area

with more extensive infiltration into the surrounding cortical bone.

There is no evidence of calcification in the high grade portion of the

lesion and it typically breaks out through the cortex and into the

subperiosteal space.

The prognosis for this variant of chondrosarcomqa is extremely

poor, most patients dying from metastatic disease within one or two

years after the diagnosis is established. Adjavent chemotherapy or

radiation therapy is not very effective, mainly because of the older

age group in which the tumor occurs. The primary treatment

modality is wide surgical resection.

CLASSIC

Case #127

44 year male

dedifferentiated

chondrosarcoma

proximal femur

2 years later with

increased size

Coronal T-1 MRI

tumor

Axial T-2 MRI

tumor

Photomic at juncture of high and low grade tumor

Low grade chondrosarcoma portion

High grade OGS portion

osteoid

Case #127.1

63 yr male with recent

hip fracture

Dedifferentiated chondrosarc

Bone scan

Cor T-1

Sag T-1 STIR

Axial PD

Axial PD

Surgical resection

Rconstruction completed

Post op X-ray

Case #687

73 year female

dedifferentiated

chondrosarcoma

distal femur

high

grade

low

grade

Lateral view

high grade

low grade

Bone scan

Coronal T-1 MRI high

low

Resected distal femur cut in path lab

high grade

low grade

Photomic showing low grade left & high grade right

Low grade chondrosarcoma

High grade OGS

osteoid

Post op x-ray with

prosthetic recon

Case #688

33 year female

dedifferentiated

chondrosarcoma

distal femur

Reconstruction with Compress system after wide resection

400 lbs pressure

Completion of rotating hinge arthroplasty

Immediate post op x-ray

X-ray at 2 months showing

early callous formation

Early osseointegration

at 5 months

spindle

anchor

plug

X-ray at one year

Stable osseointegration

at 5 years with no signs

of stress shielding

AP view

Lateral view

anterior

cortex

10 years post op

14 years post op

Case #688.1 Dedifferentiated chondrosarcoma

89 year male with mild knee pain 3 months

Coronal T-1 T-2 Sagittal T-2

Axial

T-1 T-2

Gad

Immediate Post Op x-rays

Case #689

42 year female

dedifferentiated

chondrosarcoma

pelvis

Bone scan

Sagittal T-1 MRI

tumor

post column

acetabulum

Axial T-1 MRI

Coronal T-2 MRI

tumor

Low power photomic

Low grade chondrosarcoma

High grade portion

Internal hemipelvectomy reconstruction

recon plate

Post op x-ray

Case #689.1

42 year male with right hip pain for 3 months

Bone scan

CT scan

Cor T-1 STIR

Gad Gad

Axial T-1 T-2

T-2 Gad

Clear Cell

Chondrosacoma

Clear Cell Chondrosarcoma

The clear cell chondrosarcoma is one of the rarest variants of the

chondrosarcoma. It is found more commonly in males than females

between the ages of 20 and 50 years. The most common location

for this tumor is in the femoral head. Radiographically the clear cell

chondrosarcoma has the appearance of a lytic lesion in the epiphysis,

similar to the chondroblastoma in a younger age group for which it

is frequently misdiagnosed. It has a geographic pattern with central

stippled calcification similar to that of a chondroblastoma. Histo-

locally it also has the appearance of a chondroblastoma with the

presence of benign macrophages and polyhedral stem cells with a

clear cell chicken wire appearance. But in some areas one will see

evidence of a low grade chondrosarcoma in which giant cells are not

seen, clearly separating it from the chondroblastoma.

The treatment for this condition consists of a wide resection which

in the femoral head would be a transcervical resection and replace-

ment with a bipolar prosthesis. If the lesion is treated by simple

curettement, the recurrence rate is quite high compared to the

chondroblastoma. The chance for pulmonary metastasis is very

unlikely and local recurrence is rare following a wide resection.

CLASSIC Case #128

25 year male with clear cell chondrosarcoma femoral head

Low power photomic

Higher power showing clear cells

Case #690

51 year male with clear cell chondrosarcoma femoral head

Frog leg lateral

Post op x-ray following

head & neck resection

and total hip replacement

Case #690.1

Post reduction

Acute pathologic fracture left shoulder in 43 yr female

Clear cell chondrosarcoma

CT scan

Cementation PO

Case #690.1

20 year female with clear cell chondrosarc prox tibia

Lateral view

Coronal T-1 MRI

tumor

Sagittal T-1 MRI

tumor

Mesenchymal

Chondrosarcoma

Mesenchymal Chondrosarcoma

The mesenchymal chondrosarcoma is another rare variant of the

chondrosarcoma. It consists of low grade chondrosarcoma com-

ponents with an infiltration of primitive mesenchymal cells giving

it the histological appearance of a Ewing’s sarcoma or a hemangio-

pericytoma. It can be seen in soft tissue as well as bone in young

adults, more often in females. The most common location is in the

jaw, followed next by the spine or ribs, with a very few cases seen

in long bones. Because of the high grade component of this lesion,

it is treated as a high grade sarcoma with adjavent chemotherapy

and radiation therapy along with a wide resection if possible.

Despite this aggressive program of treatment, the prognosis is very

poor because of a high incidence of pulmonary metastases and

local recurrence.

CLASSIC

Case #129

34 year female

mesenchymal

chondrosarcoma

LD spine & paraplegia tumor

Sagittal MRI

Photomic showing low grade chondroid portion

High grade round cell portion of tumor

Case #691

36 year male with mesenchymal chondrosarc humeral head

tumor

CT scan

Post op x-ray

following humeral

head resection and

prosthetic recon

Cartilagenous

Pseudotumors

Case #692

8 year female with multi focal TBc looking like Ollier’s

Pseudotumor

Geographic lesions in both elbows

Photomic showing tuberculous granuloma

Langhans giant cell

Case #693

10 year child

TBc granuloma

proximal tibia looking

like chondroblastoma

Case #694

54 year female with tumoral calcinosis looking like chondrsarc

hip

Sagittal T-1 MRI

Axial T-1 MRI

Amorphous calcium

phosphate flowing

from biopsy site

Resected specimen cut in path lab

Photomic showing heavy calcifcation

Case #695

64 yr male with giant bone island looking like chondrosarc

CT scan

Case #696

55 yr female with geode(DOA) looking like chondrosarc

AP x-ray hip

Axial CT scan

Sagittal CT scan

Bone scan

Coronal T-1 MRI

Axial proton density MRI

Axial T-2 MRI

Case #697

52 yr female with bone infarct looking like enchondroma

Sagittal T-1 MRI

knee joint

Case #697.1 Sag & Cor T-1

74 yr female with tender lump mid pretibial area for 1 year

Bone infarcts

Cor T-2

Gad

Gad

Axial T-1

T-2

Axial T-2

Case #698

45 yr female with epiphyseal infarct looking like

chondroblastoma

Case #699

77 year female

rheumatoid arthritis

shoulder looking like

chondrosarcoma

proximal humerus

AP x-ray shoulder

Bone scan

CT scan

Another CT cut

Axial T-1 MRI

Axial T-1 MRI

Sagittal T-2 MRI

Coronal T-2 MRI

Resected proximal

humerus specimen

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