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Transcript of Volume 3
Volume 3
Osteosarcoma Variants
Hemorrhagic osteosarcoma------------Case 110 & 499-503
Parosteal osteosarcoma-----------------Case111 & 504-510
Periosteal osteosarcoma----------------Case 112 & 511-517
Pagetic sarcoma-------------------------Case 113 & 518-528
Low grade intramedullary OGS------Case 114 & 528.1-530
Radiation induced OGS---------------Case 115 & 531-537
Multicentric osteosarcoma------------Case 116 & 538-542
Soft tissue osteosarcoma--------------Case 118 & 543-545
Intracortical osteosarcoma------------Case 119 & 546-547
Osteogenic
Sarcoma
Variants
Hemorrhagic
Osteogenic
Sarcoma
Hemorrhagic (Telangiectatic) Osteosarcoma
The hemorrhagic (OGS), an extremely lytic and hemorrhagic
variant of the osteosarcoma, presents in the same age group and
location as a classic osteosarcoma but has a radiographic
appearance almost identical to that of an aggressive aneurysmal
bone cyst, making for a very difficult differential consideration
for the radiologist. At the time of biopsy the tumor is very
hemorrhagic and has the gross appearance of an aneurysmal
bone cyst. Even microscopically, many areas of the hemorrhagic
OGS will have the appearance of an aneurysmal bone cyst with
only an occasional mitotic figure. For this reason, it is very
important for the surgeon who performs the biopsy to obtain
an adequate specimen with good sampling by means of an open
biopsy as apposed to a simple needle biopsy. The microscopic
features of the hemorrhagic OGS is a large number of benign-
appearing giant cells and thus the terminology “giant cell rich”
osteosarcoma that is used by many pathologists. There is very
little evidence of osteoblastic acitivity in the hemorrhagic OGS
and, because it is so lytic in character, it frequently presents with
a pathologic fracture early in the course of the disease and with that
come potential problems for the treating orthopedic surgeon who
must deal with the major contamination that occurs during the
fracture. Because of the possible complications, one might consider
an early limb salvage procedure before the fracture occurs.
It was once felt that the prognosis for the hemorrhagic OGS
was worse than that of the classic OGS because of its lytic dest-
uctive nature. However, since the advent of systemic chemotherapy,
the prognosis for survival is no different than for a classic OGS.
CLASSIC
Case #110
23 year male
hemorrhagic OGS
proximal humerus
Aneurysmal lesion
Coronal T-1 MRI
hemorrhagic
tumor
Coronal T-1 MRI
thru path fracture
tumor
Resected tumor cut in path lab
Photomic showing giant cells and malignant cells
Photomic showing hemorrhagic response
blood
osteoid
Post op x-ray with
alloprosthetic
reconstruction
Neer
allograft
18 year followup x-rays
Case #499
15 year male
hemorrhagic OGS
distal femur
Lateral view
Bone scan
Sagittal T-2 MRI
tumor
hemorrhagic
tumor
Coronal T-2 MRI
Axial T- 2 MRI
tumor
Photomic
blood
3 yrs post op Compress
total knee reconstruction
CPS
osseo-
integration
Case #500
19 year male
hemorrhage OGS
proximal femur
Looks like ABC
Lateral view
Initial biopsy reveals aneurysmal bone cyst
6 weeks later
shows lysis of
outer shell
Repeat biopsy
reveals
hemorrhagic OGS
Hip disarticulation specimen
tumor
femoral
head
2nd biopsy Photomic
Case #501
6 year female
path fracture thru
unicameral bone cyst
Lateral view
cystic
lesion
7 weeks after
steroid injection
cyst
1 month later and
progressive lytic
destruction
Biopsy here shows hemorrhagic OGS
Case #501.1
19 year old male with
acute onset of pain 2 wks
ago in right hip
Telangiectatic OGS
PO 1 mo
2 mo 3 mo
Cor T-1 T-2
Axial T-1 T-2
Gad
Sag T-2 Gad
Case #502
4 year male
looks like
unicameral bone cyst
cystic
lesion
Progressive lysis
after steroid injection
2 months later
with progressive
lysis and looking
malignant
Biopsy reveals hemorrhagic OGS
Clinical appearance before shoulder disarticulation
Case #503
17 year female
hemorrhagic OGS
C-3
AP view
CT scan
Photomic
6 years later with
spontaneous fusion
and no tumor
Parosteal
Ostogenic
Sarcoma
Parosteal Osteosarcoma
The parosteal (OGS) is a low grade variant arising from the surface
of a long bone that presents as an exophytic mass with dense fibro-
osseous tissue. It carries an excellent five year survival prognosis
of 85% and accounts for about 4% of all osteosarcomas. This
tumor has very little, if any, medullary involvement which clearly
separates it from the classic OGS. It is seen more commonly in
females than males and is found in a slightly older age group
than the classic OGS. By far the most common location for this
tumor is in the posterior aspect of the distal femur where it is
frequently presents with minimal symptoms of pain but with a
palpable tumor mass that might have been present many years
before medical advise was sought. Histologically, this tumor has a
very low mitotic index and in many cases can be confused with
a normal healing fracture callous with occasional areas of cartilage
being seen. Because this tumor is extremely low grade, it is not
responsive to adjuvant therapy such as chemotherapy or
radiation therapy. The treatment consists of a wide surgical
resection that must have safe margins, otherwise the recurrence
rate will be quite high. Recurrence can occur 10 to 15 years after
the surgery. In many cases the lesion can be resected without
sacrificing the adjacent joint, but in larger lesions the best
approach is a total joint replacement similar to that used for the
classic OGS.
CLASSIC
Case #111
32 year male
parosteal OGS
distal femur
AP view
Bone scan
Sagittal T-1 MRI
Sagittal STIR MRI
tumor
Axial T-1 MRI
Axial STIR MRI
tumor
Photomic
Higher power
Case #504
18 year male
parosteal OGS
distal femur
AP view
tumor
Bone scan
Axial T-2 MRI
tumor
Sagittal T-2 MRI
tumor
Macro section
tumor
Photomic
Compress total knee reconstruction 2 years later
osseointegration
10 years later with
recurrence as a high
grade dedifferentiated
parosteal OGS tumor
Another view
tumor
Photomic of recurrence
Close up of
osseointegration of
Compress implant
Case #505
32 year male
parosteal OGS
proximal humerus
tumor
Axillary view
tumor
CT scan
tumor
Amputation specimen cut in path lab
Photomic
Case #506
25 year male
parosteal OGS
distal femur
Distal femoral
resection specimen
tumor
Cut specimen
in path lab
tumor
fatty
marrow
Case #507
13 year male
parosteal OGS
mid femur
AP view
Lateral view
CT scan
Segmental resection specimen
Autoclaved bone replaced with IM nail fixation
Post op x-ray
2 years later
autoclaved
bone
Case #508
17 year male with parosteal OGS mid tibia
Lateral x-ray
CT scan
tumor
Bone scan
Segmental resection
mid tibial lesion
biopsy
site
Surgical specimen cut in path lab
tumor
Allograft reconstruction over IM nail
X-ray 1 year later
Case #509
41 year female
parosteal OGS
humerus
CT scan
Resected cut specimen in path lab
tumor
Case #509.1
10/06 3/07
17 year male with football injury 9/06
Parosteal OGS pseudotumor M.O.
Sag T-1 Sag Gad
Axial T-1
Axial Gad
Case #510
32 year female with high grade parosteal OGS femur
Macro section
tumor
Photomic
Periosteal
Osteogenic
Sarcoma
Periosteal Osteosarcoma
The periosteal osteosarcoma is another surface type OGS that
tends to be low grade to intermediate with potential for pulmonary
metastasis in about 25% of cases. It accounts for 2% of all OGS’s
and, compared to the parosteal OGS, has a much higher percentage
of cartilagenous tissue in the tumor to the point where it can look
like a periosteal chondroma but with a much higher mitotic index.
One must find a few areas of osteoid formation to classify this as
a periosteal OGS. It is seen typically in the second decade of life
and is slightly more common in females than males. It arises from
long bones, typically the tibia or femur, and has a higher incidence
in diaphyseal bone than does OGS. Like the parosteal OGS, this
lesion is treated by aggressive wide local resection that often can
spare the adjacent joint. In most cases chemotherapy is not utilized
unless the clinical picture is more aggressive than usual.
CLASSIC Case #112
15 year female with periosteal OGS tibia
CT scan
Sagittal CT scan
tumor
Axial T-2 MRI
Photomic
Post op x-ray following
wide resection and
allograft reconstruction
Case #511
30 year male with periosteal OGS prox tibia
CT scan
Sagittal T-2 MRI
Axial T-1 MRI
tumor
edema
Wide resection
proximal tibia tumor
bulge
Cut specimen
in path lab
tumor
Photomic
Proximal tibia resected ready for reconstruction
Post op x-ray with
alloprosthetic
reconstruction
TKA
allograft
Case # 512
9 year female
periosteal OGS
tibia
AP x-ray
Lateral view
Cut specimen in
path lab following
AK amputation
Photomic
Higher power
Case #513
14 year male
periosteal OGS
Sagittal T-2 MRI
Axial T-1 MRI
Axial T-2 MRI
Case #514
26 year female
periosteal OGS
distal femur
Lateral view
X-ray 10 years
following wide
resection and cemented
prosthetic reconstruction
stress
shielding
Case #515
12 year female
periosteal OGS
tibia
Bone scan
Axial T-1 MRI
Photomic
Case #516
15 year male
periosteal OGS
distal tibia
CT scan
Bone scan
Photomic
Case #517
39 year female
periosteal OGS
pseudotumor
In fact is a Nora’s
lesion or
bizarre parosteal
osteochondromatous
proliferation (BPOP)
Bone scan
CT scan
Axial Gad contrast MRI
edema
Sagittal PD
Sagittal T-2 MRI
edema
Axial gad contrast MRI
Pagetic Sarcoma
Pagetic Sarcoma
There are multiple diseases of the skeletal system that can result
in a secondary form of OGS most likely brought about by a second
mutation at a later age in a patient with chronic benign disease.
These diseases include Paget’s disease, osteoblastoma, fibrous
dysplasia, benign giant cell tumor of bone, bone infarcts, and
chronic osteomyelitis. The most common of this group is Paget’s
disease, a non-specific inflammatory osteomyelitis of bone seen
in older patients that may be induced by a virus infection. Approx-
imately 1% of patients with Paget’s disease can go on to Pagetic
OGS which accounts for 3% of all OGS. The most common
location for this secondary form of OGS is in the humerus,
followed next by the pelvis and femur. The patients typically have a
long history of dull, aching pain from their inflammatory Paget’s
disease but then suddenly develop an acute new pain in the area of
the older pain with x-ray evidence of recent lysis and destruction
of old Pagetic reactive bone. The prognosis for survival in this
secondary form of OGS is extremely poor with only about 8%
surviving, mainly because the older age group in which the disease
occurs make it impractical to implement the aggressive protocols
used in younger age groups.
CLASSIC Case #113
80 year female with Pagetic sarcoma pelvis
tumor
Bone scan
Axial T-2 MRI
tumor
Photomic
osteoid
Post op internal hemipelvectomy
Case#518
83 year female
Pagetic sarcoma
pelvis
tumor
CT scan
tumor
Another CT cut
tumor
Photomic
Case #519
85 year female with Paget’s disease pelvis
Same disease in
lumbar spine
Same disease
in skull
Same disease in tibia
Advancing osteolytic wedge
Same patient with
Pagetic sarcoma
humerus
tumor
old Paget’s
new
Macro section
from amputation
specimen tumor
Photomic
Post op x-ray following forequarter amputation
Case # 520
73 year female
Pagetic sarcoma skull
ready for resection
Lateral view of skull
Occipital view
Tangential view
tumor
Resected specimen cut in path lab
Photomic
Case #521
82 year male
Pagetic sarcoma
distal humerus
old Paget’s
with prior
fracture
Close up of new tumor
tumor
Photomic
Case #522
80 year female
Pagetic sarcoma
distal humerus
Case #523
84 male with multi focal Pagetic sarcoma
femur
humerus
Case #524
83 year male
Pagetic sarcoma
femur
Case #525
60 year male
Pagetic sarcoma
femur
Lateral view
Photomic
Photomic
Case #526
78 female
Pagetic sarcoma
proximal tibia
Lateral view
tumor
Case #527
92 year male
Pagetic sarcoma tibia
Case #528
78 year female
Pagetic sarcoma
lumbar spine
Low Grade
Intramedullary
Osteogenic
Sarcoma
Low Grade Intramedullary OGS
Low grade intramedullary OGS is another rare low grade fibro-
osseous variant of OGS that is unique because it is totally confined
within the cortical anatomy of a long bone, most typically around
the knee joint. It is found in an older age group than the classic
OGS and is typically seen between the ages of 15 and 55 years;
it affects males and females equally. The radiologic picture is that
of a diffuse sclerotic change within the metaphysis of the long
bone with no periosteal response or lytic destruction of the cortical
anatomy. The smoky appearance of metaphyseal bone suggests
the diagnosis of chronic osteomyelitis or perhaps fibrous dysplasia.
Microscopically, the tumor has a histological appearance similar
to parosteal OGS and because of this carries the same excellent
prognosis for survival as we see in parosteal sarcoma. Likewise,
treatment is similar without the use of chemotherapy or radiation.
These lesions must be treated with complete wide resection that
frequently involves a TKA, similar as in the classic OGS.
CLASSIC
Case #114
63 year female
intramedullary OGS
distal femur
Lateral view
Bone scan
CT scan
tumor
Macro section from
resected specimen
tumor
Photomic
Photomic
Case #528.1
51 year female
low grade
intramedullary OGS
distal femur
tumor
Bone scan
Coronal T-1 MRI
tumor
Axial T-1 MRI
tumor
Resected distal femur cut in path lab
tumor
Photomic
Case #529
32 year female
low grade
intramedullary OGS
distal femur
tumor
Lateral view
CT scan
Photomic
Case #530
56 year male
low grade
intramedullary OGS
distal tibia
tumor
Lateral view tumor
Bone scan
Photomic
Radiation-induced
Osteogenic
Sarcoma
Radiation-induced Osteosarcoma
One of the most malignant forms of OGS is the secondary type
induced by radiation therapy, usually over 3000 rads, for some
type of either benign or malignant disease process in the past.
One of the most common types of radiation-induced OGS is
seen in patients with breast cancer who receive local radiation
following radical mastectomy and than develop OGS in the
shoulder girdle area. Other malignant diseases that can result in
OGS after radiation therapy include Ewing’s sarcoma and
lymphomas. Benign diseases that can result in OGS from
radiation therapy include GCT,ABC, and fibrous dysplasia. The
average delay for the occurrence of secondary OGS is 15 years,
with a range from 3 to 55 years. The prognosis for this variant is
extremely poor, similar to Pagetic OGS. It has a very high rate
of metastasis to the lung for which chemotherapy is not very
effective.
CLASSIC
Case #115
33 year female
radiation-induced
sarcoma scapula
Widely resected specimen cut in path lab
tumor
Close up
tumor
scapula
Photomic
Higher power
Post op x-ray following scapular wing resection
Case #531
35 year female with radiation sarcoma prox femur
tumor
prior radiation treatment for Hodgkin’s 20 yrs ago
Frog leg lateral
tumor
Shortly after with
pathologic fracture
Biopsy photomic
tumor
Higher power
Case #532
72 year male
radiation sarcoma pelvis
Prior radiation therapy
for prostate cancer
3 years before
Another view at a
different date with
hip dislocation
Photomic
Case #532.1
79 yr male with prior prostate CA radiation therapy
and now presents with radiation OGS
Radiation induced OGS
Coronal Anterior CT Posterior CT
L Sagittal CT scan R Sagittal CT scan
Low axial CT
cut thru L hip
showing large tumor
Upper CT cut thru
SI area showing
tumor R post ilium
Metastatic disease seen on chest x-ray
Case #533
56 year female with radiation sarcoma scapula
Prior history of radiation for breast cancer
Oblique view
Bone scan
Photomic
Case #534
63 year female with radiation sarcoma scapula
with prior radiation treatment for breast CA 12 yrs ago
tumor
Case #535
44 year female with
radiation sarcoma
proximal humerus 2nd
to prior radiation for
breast cancer tumor
Photomic with radiation OGS
Case #536
76 year male
radiation sarcoma
femur
Prior history of
radiation therapy
for soft tissue tumor
10 years ago
Bone scan
Photomic radiation OGS
Case #537
Elderly M.D. with long
history working under
X-ray fluoroscope
Now skin cancer and
radiation sarcoma
index finger
X-ray of index
finger sarcoma
tumor
Photomic radiation sarcoma
Multicentric
Osteogenic
Sarcoma
Multicentric Osteosarcoma
The multicentric variant of OGS is an extremely rare variant
occurring in approximately 1% of all OGS. It has two distinct
categories: (1) Synchronous multicentric OGS occurring in child-
hood and adolescence. This is the more severe variant, considered
to be extremely high grade with a very poor prognosis associated
with it. This form presents with multiple sclerotic lesions seen in a
fairly symmetrical fashion in long bones, mostly in the lower
extremities and because of the heavy tumor burden associated
with multiple lesions throughout the skeleton, the alkaline phos-
phatase is frequently elevated. (2) Metachronous multicentric OGS
occurring mainly in adults is less aggressive than the synchronous
form seen in children, presenting usually with a solitary lesion.
Then, later on, more lesions develop that are considered multi-
focal in nature. The possibility of metastasis can not be ruled out.
These forms of OGS are quite resistant to chemotherapy and
surgical treatment is frustrating because of the multi focal disease.
CLASSIC
Case #116
8 year female
multicentric OGS
Close up distal femur
Lateral view
Bone scan
Close up
bone scan
Upper body
bone scan
Coronal T-1 MRI
Another coronal cut
T-1 MRI
Sagittal T-1 MRI
Photomic
Another photomic
Case #538
18 year female
multicentric OGS
pelvis and femur
Gad contrast coronal MRI
tumor
tumor
Another Gad contrast cut
Axial T-2 MRI
pelvic
tumor
Recon plate placed across pelvic ring surgical defect
Internal Hemipelvectomy
Placement of air screws just prior to cementation
Placement of cement around screws and plate
cement
Constrained total hip in and securing muscles
to custom proximal femoral replacement implant
total
hip
femoral implant
Outer face of resected specimen
acetabulum
ilium
tumor
bulge
Inner face
tumor
bulge
ilium
Closure
Post op x-ray
recon plate
and screws
Case #539
16 year female
multicentric OGS tumor
Proximal tibial lesion
Lateral view with
skip lesion in
distal tibia
Bone scan showing two lesions in tibia
Bone scan showing
iliac lesion
Bone scan showing
sternal lesion
Photomic from tibial biopsy
Proximal tibial
resection and
total knee
reconstruction
tumor
bulge
Proximal tibial
prosthesis in position
ready for relocation
and closure
Reconstruction
completed and
ready for closure
Case #540
Multicentric OGS
femur and sacrum
20 year male
Lateral view
Coronal T-1 MRI
showing tumor at
both ends of femur
Sagittal T-1 MRI
distal femur
tumor
Axial T-2 MRI distal femur
tumor
Another axial T-2 MRI
Bone scan
Coronal T-1 MRI
Axial T-1 MRI
Coronal gad contrast MRI showing sacral lesion
tumor
Photomic from femoral biopsy
Case #541
10 year female with multicentric OGS femur and tibia
Lateral view
tumor
tumor
skip
lesion
AP view femur
Coronal T-2 MRI
distal femur
tumor
Sagittal T-2 MRI
distal femur
tumor
tibial
lesions
Coronal T-1 MRI
knee joint
tumor
tumor
Coronal T-1 MRI showing multicentric involvement
tumor
Case #542
15 year male with multicentric OGS tibia and femur
tumor
Coronal T-1 MRI
tumor
tumor
Coronal T-2 MRI
tumor
Sagittal T-1 MRI
tumor
Axial T-2 MRI view of distal femur
tumor
Soft Tissue
Osteogenic
Sarcoma
Soft Tissue Osteosarcoma
OGS can be seen in soft tissue outside the skeletal system. It
accounts for 4% of all OGS and is typically in large muscle groups
around the pelvis and thigh area. It occurs most often in patients
over 40 years of age and hits males and females equally. Soft
tissue OGS, with its mature appearing bone in the central area of
the lesion and aggressive, poorly mineralized tissue at the
periphery, must be differentiated from myositis ossificans, which
has a typical zonal pattern with peripheral maturation of bone
formation. As with any soft tissue sarcoma, the treatment consists
of wide local resection. Because of the poor prognosis, worse
than that of bone osteosarcoma, systemic chemotherapy is utilized
extensively as one would use for a typical medullary OGS.
CLASSIC
Case #118
67 year male
soft tissue OGS
calf
tumor
AP view
Sagittal T-1 MRI
tumor
Axial T-1 MRI
Cut surgical specimen in path lab
Photomic
Case #543
76 year female
soft tissue OGS
calf
Lateral view
CT scan
Bone scan
Axial T-1 MRI
Sagittal T-1 MRI
tumor
Photomic
Case #544
60 year female with
soft tissue OGS leg
Oblique view
Bone scan
Case #545
63 year male
soft tissue OGS
hand tumor
Lateral view
Axial T-1 MRI
tumor
Axial T-2 MRI
tumor
Coronal T-2 MRI
tumor
Multiple pulmonary mets
Intracortical
Osteogenic
Sarcoma
Intracortical Osteosarcoma
The intracortical OGS is perhaps the rarest variant of OGS with
only 14 cases described in the world literature since 1960. It
occurs between the ages of 10 and 47 years, equally between
males and females, and is seen most typically in the femur or
tibia as a metadiaphyseal lesion with a radiographic appearance
very similar to that of osteoid oasteoma. The prognosis is usually
quite good with a total of three deaths in the world literature. It
is usually treated by wide resection without chemotherapy. A
few cases are higher grade and carry a poor prognosis similar
to the classic OGS.
CLASSIC Case #119
42 year female with intracortical OGS femur
Bone scan
Axial PD MRI
Sagittal T-2 MRI
Early biopsy photomic
X-ray 18 months
after curettement
with recurrence
Bone scan at time of recurrence
Axial Gad contrast MRI same time
Sagittal PD MRI
same time
tumor
Unicortical segmental wide resection
tumor
Photomic of resected specimen
Post op x-ray following
unicortical resection
and allograft recon
allograft
Sagittal PD & T-2 MRI 18 months later with met to C-spine
tumor
PD T-2
Case #546
43 year female
intracortical OGS
distal femur
Lateral view
Sagittal T-1 MRI
Sagittal T-2 MRI
tumor
Biopsy photomic
Photomic
Case #547
47 year female
intracortical OGS
humerus
Lateral view
CT scan
Sagittal T-1 MRI
tumor
Post op x-ray after
wide resection and
allograft recon