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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 are 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 these 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 that of a classic OGS.
CLASSIC
Case #110




23 year male
hemorrhagic OGS
proximal humerus



Aneurysmal lesion
hemorrhagic
                  tumor
Coronal T-1 MRI
Coronal T-1 MRI      tumor
thru path fracture
Resected tumor cut in path lab
Photomic showing giant cells and malignant cells
osteoid


           blood




Photomic showing hemorrhagic response
Neer
                            allograft
Post op x-ray with
alloprosthetic
reconstruction
18 year followup x-rays
Case #499




15 year male
hemorrhagic OGS
distal femur
Lateral view
Bone scan
tumor

Sagittal T-2 MRI
hemorrhagic
 tumor




Coronal T-2 MRI
tumor




Axial T- 2 MRI
blood




Photomic
osseo-
                              integration
3 yrs post op Compress
total knee reconstruction


                            CPS
Case #499.1                    Hemorrhagic OGS




 22 year male with a 3 month history of right knee pain
Cor T-1   PD FS   Gad
Sag
PD
Axial PD FS   Gad
Resection 16 cm distal femur and Compress recon
PO x-ray
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
femoral
                                head

         tumor




Hip disarticulation specimen
2nd biopsy Photomic
Case #501




6 year female
path fracture thru
unicameral bone cyst
cystic
lesion




         Lateral view
7 weeks after       cyst
steroid injection
1 month later and
progressive lytic
destruction
Biopsy here shows hemorrhagic OGS
Case #501.1                  Telangiectatic OGS




 19 year old male with
 acute onset of pain 2 wks
 ago in right hip
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
                       cystic
looks like
                       lesion
unicameral bone cyst
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
tumor




Sagittal STIR MRI
Axial T-1 MRI
tumor




Axial STIR MRI
Photomic
Higher power
Case #504




 18 year male
 parosteal OGS
 distal femur
tumor
AP view
Bone scan
tumor




        Axial T-2 MRI
tumor




Sagittal T-2 MRI
tumor




   Macro section
Photomic
osseointegration




   Compress total knee reconstruction 2 years later
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      tumor
proximal humerus
tumor




Axillary view
tumor




CT scan
Amputation specimen cut in path lab
Photomic
Case #506




25 year male
parosteal OGS
distal femur
Distal femoral
resection specimen
                     tumor
tumor
Cut specimen
in path lab


                       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
tumor




CT scan
Bone scan
biopsy
                      site
Segmental resection
mid tibial lesion
tumor




Surgical specimen cut in path lab
Allograft reconstruction over IM nail
X-ray 1 year later
Case #509




41 year female
parosteal OGS
humerus
CT scan
tumor




Resected cut specimen in path lab
Case #509.1           Parosteal OGS pseudotumor M.O.
                    10/06                              3/07




              17 year male with football injury 9/06
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
tumor




Sagittal CT scan
Axial T-2 MRI
Photomic
Post op x-ray following
wide resection and
allograft reconstruction
Case #112.1        Periostel OGS




        17 yr male with tender lump on tibia for 8 mos.
Sag T-1   Gad
Axial T-1   Gad
Case #511




      30 year male with periosteal OGS prox tibia
CT scan
Sagittal T-2 MRI
edema




     tumor




Axial T-1 MRI
Wide resection
proximal tibia
                 tumor
                 bulge
tumor
Cut specimen
in path lab
Photomic
Proximal tibia resected ready for reconstruction
Post op x-ray with
alloprosthetic                   TKA
reconstruction



                     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
stress
                            shielding

X-ray 10 years
following wide
resection and cemented
prosthetic reconstruction
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
                       Nora’s lesion


39 year female
periosteal OGS
pseudotumor

In fact is a Nora’s
lesion or
bizarre parosteal
osteochondromatous
proliferation (BPOP)
Bone scan
CT scan
edema




    Axial Gad contrast MRI
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




          tumor




      80 year female with Pagetic sarcoma pelvis
Bone scan
tumor




        Axial T-2 MRI
osteoid




Photomic
Post op internal hemipelvectomy
Case#518

                  tumor



83 year female
Pagetic sarcoma
pelvis
tumor




    CT scan
tumor




Another CT cut
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
old Paget’s

Same patient with
Pagetic sarcoma
humerus

                       new
                      tumor
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
                  old Paget’s
                  with prior
                  fracture


82 year male
Pagetic sarcoma
distal humerus
tumor

Close up of new tumor
Photomic
Case #522




80 year female
Pagetic sarcoma
distal humerus
Case #523

            femur

                                        humerus




      84 male with multi focal Pagetic sarcoma
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
tumor




        CT scan
tumor
Macro section from
resected specimen
Photomic
Photomic
Case #528.1




51 year female       tumor
low grade
intramedullary OGS
distal femur
Bone scan
Coronal T-1 MRI

                  tumor
tumor




Axial T-1 MRI
tumor




Resected distal femur cut in path lab
Photomic
Case #529




32 year female       tumor
low grade
intramedullary OGS
distal femur
Lateral view
CT scan
Photomic
Case #530




56 year male
low grade            tumor
intramedullary OGS
distal tibia
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
tumor




Widely resected specimen cut in path lab
scapula




tumor




        Close up
Photomic
Higher power
Post op x-ray following scapular wing resection
Case #531




                                          tumor




   35 year female with radiation sarcoma prox femur
   prior radiation treatment for Hodgkin’s 20 yrs ago
Frog leg lateral


                   tumor
Shortly after with
pathologic fracture
tumor




Biopsy photomic
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                Radiation induced OGS




  79 yr male with prior prostate CA radiation therapy
        and now presents with radiation 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




                            tumor




      63 year female with radiation sarcoma scapula
  with prior radiation treatment for breast CA 12 yrs ago
Case #535




44 year female with
radiation sarcoma
proximal humerus 2nd
to prior radiation for   tumor
breast cancer
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 #116.1              Multicentric OGS
              Cor STIR                      Bone scan




              13 yr male with left knee pain for last 3 mos
Sag T-1   PD   Gad
Axial T-1   T-2




Gad
Sag T-1   T-1
CT lung
Case #538




18 year female
multicentric OGS
pelvis and femur
tumor



tumor




Gad contrast coronal MRI
Another Gad contrast cut
pelvic
tumor




         Axial T-2 MRI
Internal Hemipelvectomy




Recon plate placed across pelvic ring surgical defect
Placement of air screws just prior to cementation
cement




Placement of cement around screws and plate
femoral implant




   total
   hip


 Constrained total hip in and securing muscles
to custom proximal femoral replacement implant
acetabulum




             tumor
             bulge           ilium




Outer face of resected specimen
tumor
          bulge
ilium




        Inner face
Closure
recon plate
                and screws




Post op x-ray
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     tumor
total knee        bulge
reconstruction
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
tumor




Axial T-2 MRI distal femur
Another axial T-2 MRI
Bone scan
Coronal T-1 MRI
Axial T-1 MRI
tumor




Coronal gad contrast MRI showing sacral lesion
Photomic from femoral biopsy
Case #541




  10 year female with multicentric OGS femur and tibia
tumor




        Lateral view
skip
                        lesion



AP view femur




                tumor
tumor
Coronal T-2 MRI
distal femur
tumor

Sagittal T-2 MRI
distal femur



                           tibial
                           lesions
tumor
Coronal T-1 MRI
knee joint




                   tumor
tumor




Coronal T-1 MRI showing multicentric involvement
Case #542




                  tumor




   15 year male with multicentric OGS tibia and femur
tumor




Coronal T-1 MRI



                   tumor
Coronal T-2 MRI


                  tumor
Sagittal T-1 MRI

                   tumor
tumor




Axial T-2 MRI view of distal femur
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




                  tumor
67 year male
soft tissue OGS
calf
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
tumor




Axial T-1 MRI
tumor




Axial T-2 MRI
tumor




Coronal T-2 MRI
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
tumor
Sagittal PD MRI
same time
tumor




Unicortical segmental wide resection
Photomic of resected specimen
Post op x-ray following   allograft
unicortical resection
and allograft recon
PD                        T-2




                   tumor




Sagittal PD & T-2 MRI 18 months later with met to C-spine
Case #546




43 year female
intracortical OGS
distal femur
Lateral view
Sagittal T-1 MRI
tumor

Sagittal T-2 MRI
Biopsy photomic
Photomic
Case #547




47 year female
intracortical OGS
humerus
Lateral view
CT scan
tumor
Sagittal T-1 MRI
Post op x-ray after
wide resection and
allograft recon

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Osteosarcoma Variants: Hemorrhagic, Parosteal & Periosteal Types