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OSTEOSARCOMA
DR KULDEEP RATHI
GMC Patiala
DEFINITION
 It is a malignant mesenchymal tumor in which the tumor cells
produce bone matrix or osteoid.
OSTEOSARCOMA
 It is m/c non-hematologic primary malignancy of bone
 the second most common primary malignancy of bone behind multiple
myeloma
 m/c radiation associated sarcoma
 Lungs are m/c site of metastases in OS
 .
EPIDEMIOLOGY
 incidence is 2 cases per million persons per year
 peak incidence is in 2nd – 3rd decades.
 It is more frequent in tall people than in short people.
 Bones having the fastest rates of growth have the highest frequency of occurrence
 Slightly more common in males
Associated syndromes
o Hereditary form of retinoblastoma
o Li-Fraumeni syndrome (p53)
o Rothmund-Thomson syndrome (8q24)
o Beckman-Wiederman syndrome
o Paget disease or fibrous dysplasia
5
EXCITING FACTORS
Some virus like polyoma virus
Radiation >2000 rads
Chemicals – chlorantherene, Beryllium compounds
6
 Arise from multipotent mesenchymal cells
 usually in the medullary cavity of the metaphysis of a growing long tubular bone,
it may also arise on the surface of a bone, or from an extra-skeletal site.
 the majority of medullary origin are high grade, whereas surfaces OS are of lower
grade.
Origin
PATHOGENESIS
The tumor arise in the metaphysis where
the growth is more active.
The tumor originate beneath the
periosteum
After originating tumor may extend in
two directions
1 towards medulla or 2. towards sub-
periosteal area
This triangular area is known as codmans triangle.
In the medulla the bone trabeculae are destroyed
Tumor appear as irregular mass permeating
along medullary canal
At first the periosteum offers impenetrable barrier
and is only raised off the bone.
On diaphyseal side of tumor the periosteum is
often stripped for a short distance
There may be deposition of layers of new bone
parallel to shaft
GROSS PATHOLOGY
 OS are bulky tumors that are gritty,
 grayish-white in color
 areas of hemorrhage and cystic
denegation
 Destruction of cortex
 spread in medullary canal, infiltrating and
replacing marrow
 Rarely , they penetrate epiphyseal plate
or enter the joint
 There is Reactive periosteal new bone
formation
10
GROSS PATHOLOGY
MICROSCOPICALLY
High grade spindle cell tumor that
produces osteoid matrix unconnected
by cartilage
Tumor cells produce neoplastic bone -
basophilic thin trabeculae resembling
fungal hyphae or
neoplastic osteoid - eosinophilic,
homogenous, glassy with irregular
contours and osteoblastic rimming
Vascular invasion and areas of necrosis
common
MICROSCOPICALLY
May have osteoblastic, fibroblastic or
chondroblastic predominance
Osteoid in variable amount
stroma contain bizarre giant cells or acellular
Tumor cells may be spindle, oval or round of
variable size
25% of os have osteoclast-like multinucleated
giant cells
Cartilage may be mineralized, immature, myxoid
TYPES OF OSTEOSARCOMA
1. Central
a. High-grade
 Conventional
 Telangiectatic
 Small cell
 Epithelioid
 Osteoblastoma-like
 Chondroblastoma-like
 Fibrohistiocytic
 Giant cell–rich
b. Low-grade
 Low-grade central
 Fibrous dysplasia–like
2. Surface
a. Low-grade
 Parosteal
b. Intermediate-grade
 Periosteal
c. High-grade
 Dedifferentiated parosteal
 High-grade surface
3. Intracortical
4. Gnathic
5. Extraskeletal
 High-grade
 Low-grade
CONVENTIONAL OSTEOSARCOMA
The tumor cells vary from spindle to
polyhedral;
their nuclei are pleomorphic and
hyperchromatic.
Mitotic figures are easily demonstrable.
extracellular matrix that may be osseous,
cartilaginous, or fibrous in various proportions.
The production of bone directly by tumor
cells at least somewhere in the tumor is the
absolute requirement for diagnosis
high-grade tumors and need chemotherapy
abundant production of osteoid and bone matrix
in a lacy pattern into which the malignant cells
are incorporated.
TELANGIECTATIC OSTEOSARCOMA
 It resembles aneurysmal bone cyst
radiographically and histologically.
 there is asymmetric expansion of the
involved bone with radiolucent bone
destruction
 but in ABC, a thin shell of periosteal
neocortex IS not seen.
telangiectatic OS ABC
an enhancing thin peripheral rim and septa without nodularity
or osteoid matrix mineralization.
the growth is less aggressive, with expansile well-defined
encapsulated margin,
asymmetric expansion, geographic lysis of bone,
and an aggressive growth pattern with cortical
destruction and minimal peripheral sclerosis
and infiltrative margins
An eccentric radiolucent destructive lesion.
Curetting was done & histopathology showed features of ABC. but
One month later, the patient returned with pain and increasing size of mass with destruction and expansion
of the entire metaphyseal region of the right humerus.
Retrospectively, first radiograph showed a medial interrupted periosteal reaction
 At low power, telangiectatic os is
composed of multiple blood-filled
sinusoids with little solid like aneurysmal
bone cyst,
 At higher power, there is nuclear
pleomorphism and a high mitotic rate.
 there is local destruction with permeation
to adjacent marrow
highly atypical cells lining the septa in a
telangiectatic osteosarcoma not seen in ABC
presence of several benign giant cells in the
septa can be mistaken for giant cell tumor.
SMALL CELL OSTEOSARCOMA
 a rare histologic variant of osteosarcoma with histologic features
combining those of osteosarcoma and Ewing sarcoma.
 It constitutes 1% and 2% of all osteosarcomas.
 The radiologic features are not typical
permeative lytic bone destruction , a soft-tissue mass, and
periosteal reaction
sometimes with pathological fracture
 Histologically, it may be mistaken for Ewing
sarcoma/primitive neuroectodermal tumor
(PNET) because its cells are small and have
round, hyperchromatic nuclei with very little
nuclear pleomorphism.
the production of osteoid by tumor cells is not a
feature of Ewing sarcoma,
The tumor above the bone trabecula is composed of small cells dominated by round, blue
nuclei suggestive of Ewing sarcoma/primitive neuroectodermal tumor.
The tumor below the trabecula demonstrates that the tumor cells are separated by pink
osteoid matrix
AT HIGHER MAGNIFICATION
the tumor cells above the trabecula are
indistinguishable from Ewing sarcoma
the tumor cells below the trabecula are
clearly producing osteoid matrix
EPITHELIOID OSTEOSARCOMA
 an osteosarcoma in which the tumor cells are so poorly differentiated
that it is difficult to determine histologically whether the tumor is a
sarcoma or a carcinoma.
 The tumor cells often are round or
polyhedral rather than spindle-
shaped.
 Their round or ovoid enlarged nuclei
may contain 1 or more prominent
nucleoli,
 The cells may demonstrate gland-like
structures or even contain cells
arranged in papillary configurations
clusters of polyhedral cells aggregated about
central cores resembling papillary structures;
some of these contain osteoid rather than
vessels.
Higher magnification -- epithelioid round cells loosely disposed
among a few wisps of osteoid
 If tumors like these are seen in young patients, osteosarcoma
should be considered.
 In older patients, the diagnosis of a poorly differentiated
metastatic carcinoma is more likely.
OSTEOBLASTOMA-LIKE AND
CHONDROBLASTOMA-LIKE OSTEOSARCOMA
 Osteoblastoma-like osteosarcoma
produces the same sort of microtrabecular
bone lined by osteoblasts as does
osteoblastoma.
 The radiographic appearance often
suggests a malignant.
a well-circumscribed tumor.
 parameters for malignancy include
permeation of normal bone at its
interface with tumor and aneuploid
mitotic activity are seen in os
 whereas benign osteoblastic tumors
shows maturation and circumscription at
their edges.
Microtrabecular osteoid lined by osteoblast-
like cells as seen in osteoblastomas
Chondroblastoma-like osteosarcoma may be epiphyseal.
When it is epiphyseal and well circumscribed, it is a very difficult diagnosis to make because
radiologic studies suggest a benign diagnosis.
In these cases, the diagnosis must be made histologically
radiographs demonstrate a well circumscribed radiolucency centered in the distal end of femur,
CT- Showed that the lesion is confined to the bone
This type of osteosarcoma is best distinguished by its osteoid or bone formation, atypical mitotic activity, and infiltration of
adjacent inter-trabecular spaces
1. a monotonous lesion composed of uniform cells with demarcated cytoplasmic borders and with bean-shaped, ovoid nuclei
and fine, lattice-like calcifications in a chondroid background
2. more thorough sampling revealed foci of typical lace-like osteoid infiltrating cancellous bone at the periphery, diagnostic of
osteosarcoma.
GIANT CELL–RICH OSTEOSARCOMA
 About 25% of osteosarcomas contain
benign multinucleated giant cells.
 Rarely, an osteosarcoma may contain so
many benign giant cells that the
malignant elements in the background
are obscured; and the lesion may be
mistaken for a giant cell tumor.
 This is more apt to occur in the sacrum,
where giant cell tumor is much more
common
radiographs reveal a locally destructive tumor extending from
the metaphysis to the articular end of the bone.
There is internal circumscription and no periosteal reaction.
1 3
2
2. nuclear atypia and atypical mitotic
activity are clearly shown ,
1. a giant cell rich lesion with
mononuclear background;
3. Individually malignant mononuclear
cells and even polyploid giant cells within
a collagenized matrix focally having the
properties of osteoid
 the usual giant cell tumor tends to arise in skeletally mature
people and extends from the metaphyseal side of the growth
plate to the ends of the long bones.
 In the absence of a fracture, a periosteal reaction also is
uncommon in an uncomplicated giant cell tumor.
 A tumor that appears to be a giant cell tumor histologically but
arises in a skeletally immature person, should be regarded with
great suspicion and sampled widely to rule out osteosarcoma.
Specially if it is accompanied by a visible periosteal reaction.
GNATHIC OSTEOSARCOMA
Osteosarcomas of the mandible and
maxilla
usually manifest with swelling or pain.
Radiographically, they often are
radiolucent or a mixture of radiolucent
and radiodense areas.
 often are high-grade tumors
histologically,
 they give rise to uncontrolled local
disease,
 small minority of patients develope
distant metastatic disease
Histologic sections demonstrate a mostly
cartilaginous, highly cellular tumor that contains
foci of immature osteoid production.
Low-Grade Central
Osteosarcoma
unusual variant of osteosarcoma
Its microtrabecular osseous matrix
architecture in a bland fibrous stroma
bears some resemblance to fibrous
dysplasia and other benign lesions.
 but
 atypical mitotic activity may lead
to the correct diagnosis.
1.The majority shows immature, poorly formed, woven trabeculae
without appositional osteoblastic activity in a modestly cellular
fibrous as in fibrous dysplasia but
2. nuclei at higher magnification revealed mitotic activity and
atypical mitoses
 The imaging features suggests a lesion of
greater local aggressiveness than fibrous
dysplasia or other benign lesions.
 These features include indistinct
circumscription, dense sclerosis, an
interrupted periosteal reaction, or
cortical infraction.
 Although patients usually are treated
with surgery alone and their prognosis is
significantly better than in conventional
OS
radiographs demonstrate a mottled,
circumscribed lesion that resembles fibrous
dysplasia except for the very focal area of cortical
erosion of the lateral femoral metadiaphysis
Surface Osteosarcomas
 these are osteosarcomas whose
epicenters are outside the cortex of the
underlying bone.
 They usually arise in relation to the
periosteum or the cortex of the bone with
minimal or no involvement of the
medullary cavity.
 usually arises in the third and fourth
decade.
 The majority are low-grade neoplasms
with a propensity for local recurrence and
very limited capacity for distant
metastasis.
 approximately 10% of surface
osteosarcomas are high-grade tumors.
Parosteal Osteosarcoma
 This lesion is often confused with
osteochondroma and osteoma
 3rd -4th decade
 It has a unique presentation and anatomic
distribution
 75% to 80% of cases arise as densely
radiopaque masses attached to the distal
posterior femur.
 Clinically may give rise to a sensation of
fullness, prevent complete range of flexion,
and perceived as a mass through the
overlying soft tissues,
 it is unusual for the entity to be spontaneously
painful.
 Its radiodensity often is not uniform, often being
more radiodense centrally and less dense at its
periphery.
 In contrast with osteochondroma, there is no usual
continuity with the adjacent cortex, and its inside is
not in continuity with the underlying medullary
cavity
 In localized heterotopic ossification (so-called
myositis ossificans ) the ossification pattern is exactly
the opposite radiographically, with the densest
bone being outside and the least dense being
inside.
 In parosteal osteosarcoma, there is an incomplete
radiolucent line separating most of the sclerotic
mass from the underlying cortex,
 this radiolucent line corresponds to the underling
periosteum, to which the mass usually is fixed at one
point on the outside.
 An underlying periosteal reaction is very unusual
because to produce such a reaction, the tumor
would have to lift the inner cambium layer of the
periosteum away from the cortex.
 Parosteal osteosarcoma usually is not locally
aggressive enough to do this.
sclerotic mass, partially ill-defined posteriorly to the
distal femur
 Histologically, parosteal osteosarcomas
consist of streamers of bone
trabeculae that often show a high
degree of parallel orientation similar to
that observed in a periosteal new bone
reaction.
 There is histologic resemblance of this
bone formation to “flowing steel wool.”
 There is a cellular cartilage cap
external to the osseous portion of the
lesion in about 25% to 30% of cases.
flowing steel wool appearance
cartilage cap
 The histologic key to the correct
diagnosis is that a growing
osteochondroma has the same internal
development as a growing bone.
 There is a medullary cavity with fat or
hematopoietic marrow in the
intertrabecular spaces of normal bone
and osteochondroma, whereas the
intertrabecular spaces of parosteal
osteosarcomas contain cellular fibrous
tissue.
 The majority of parosteal osteosarcomas
are associated with the outer fibrous
layer of the periosteum, which does not
produce typical periosteal reactions.
Although the fibrous tissue between the bone spicules is
bland, there is no marrow evident.
A careful search in the fibrous component sometimes
reveals focal hypercellularity and early immature bone
formation
Periosteal Osteosarcoma
 Although the lesion is associated with the bone
surface, it arises between the cortex and the
cambium layer of the periosteum, so that there
often is a periosteal reaction visible
radiographically.
 There is underlying cortical thickening or
erosion
 it usually occurs along the tibial or femoral
diaphysis rather than posterior to the
metaphysis of the distal femur.
 Its histologic grade is intermediate
 rarely it may extend into the underlying cortex,
but never is extend into the underlying
endosteum
Dedifferentiated Parosteal Osteosarcoma
 histologically high-grade
 arising in the clinical setting of
recurrent parosteal osteosarcoma of
the usual low-grade variety.
 the dedifferentiation took place over
time in recurrences of well-
documented tumors that previously
were diagnosed as typical low-grade
parosteal osteosarcomas.
cloudy, radiodense tumor matrix in the soft tissues.
the portion of lateral femoral condyle that does not overlap
the patella and the upper medial metaphysis are markedly
radiolucent compared with the bone of the metadiaphysis
 Histologically, the tissue is composed of an admixture of typical
appearing low-grade parosteal osteosarcoma and high-grade
conventional parosteal osteosarcoma
 The is worse than that of low-grade parosteal osteosarcoma but
somewhat better than that of pure high-grade surface
HIGH-GRADE SURFACE
OSTEOSARCOMA
 It manifests as a surface lesion of
bone, but is high grade histologically.
 It may appear radiographically as
an ordinary low-grade parosteal
osteosarcoma with dense sclerosis.
 its local growth and aggressiveness
are more accelerated.
 patients with this disease have more
distressing symptoms and signs than
those with low-grade parosteal
osteosarcomas.

the underlying tibia shows no
gross invasion by the tumor
 Microscopically, the tumor is entirely
high grade.
 There usually is very little or no
medullary invasion by the tumor on
xrays.
a high-grade neoplasm producing wispy
osteoid
A computed tomography scan shows separation of the
mass from the underlying bone by a dark periosteal line.
Intracortical Osteosarcoma
 very rare anatomic variant of
osteosarcoma,
 a high-grade osteosarcoma entirely
confined within the bony cortex.
 manifests as an area of cortical
radiolucency with peri-lesional sclerosis.
 Depending on its size, it may be
confused with osteoid osteoma and
osteoblastoma, but it has cellular atypia
and is locally aggressive
 Histologically, there is abundant osteoid
of bone formation.
MULTIFOCAL OSTEOSARCOMA
 This extremely unusual condition affects multiple osseous sites
simultaneously at the time of presentation (synchronous form) or
 multiple skeletal sites at varying intervals (metachronous type) .
 The former variant tends to affect children and adolescents,
whereas the latter type occurs in adults.
 The childhood synchronous form is radiodense, usually symmetric,
and a high-grade sarcoma with a rapidly fatal course.
 In the adult metachronous form, after the appearance of the first
osteosarcoma, there is a disease-free interval of several months to
years followed by the development of one or several skeletal
osteosarcomas.
 These lesions are not symmetric, they may have variable degrees of
sclerosis, and their histologic features may vary from high to low
grade.
 The metachronous form have a longer survival interval than children
with the synchronous form
Synchronous Multifocal
Osteosarcoma
EXTRA-SKELETAL OSTEOSARCOMA
 These accounts for less than 2% of all
soft tissue sarcomas.
 it tends to occur in late adulthood,
 the fifth to seventh decades of life
 Most cases arise in the deep soft tissues
with a predilection for the thigh
followed by the buttocks, upper
extremities, and the retroperitoneum.
 Patients usually have an enlarging soft tissue mass, and the mass
may be painful
 Radiographs show a mass with mineralization
 Histologically, the tumors demonstrate all types of osteosarcoma
seen in bone tumors.
 The prognosis is poor in soft tissue osteosarcoma of the high-
grade type.
 patients with the low-grade variant seem to have a better
prognosis
SKELETAL DISTRIBUTION
 Distal femur
 Proximal tibia
 Proximal humerus
(sites of rapid bone growth)
 others
Metaphyseal(89%)>diaphyseal(10%)>epiphyseal(1%)
53
CLINICAL PRESENTATION
 m/c symptom -pain for several weeks or
months.
 The most common sign is a mass of variable
consistency( soft to hard) and tender.
 There may be superficial erythema, venous
distension, or other signs of hyper-vascularity.
There may be a limp, loss of function, or even
decreased range of motion
Fever, malaise or other constitutional symptoms are
not typical of osteosarcoma
COURSE
 Metastasis occur early by hematogenous rout
 Progressive pulmonary metastasis develop very early
 Process is usually fatal within 2 years
X- RAY
 Variable portions of radiopacities and
radiolucencies,
 medullary and cortical bone destruction
 wide zone of transition, permeative or
moth-eaten appearance
 aggressive periosteal reaction
 sunburst type
 Codman triangle
 lamellated (onion skin) reaction: less
frequently seen
 soft-tissue mass
 tumour matrix ossification/calcification
 The cambium layer of the inner
periosteum reacts to separation from the
cortex by producing new bone, which is
sometimes visible as an incomplete bony
shell that appears attached to the bone
surface on only one end and is open in
the middle, so-called Codman angle.
 Although periosteal new bone formation
sometimes is associated with benign
conditions, periosteal reactions
associated with malignant tumors usually
are discontinuous, implying that the
tumor growth is too rapid for periosteal
containment.
 Lesions are usually permeative
 Associated with destruction of the cancellous and
cortical elements of the bone
 Ossification within the soft tissue component, if
tumour has broken through cortex
 Borders are ill defined
Plain X-ray
59
PLAIN X-RAY
Periosteal reaction may appear as the
characteristic Codman triangle.
Extension of the tumor through the periosteum may
result in a so-called “sunburst” or “hair on end”
appearance.
QUESTION
A completely radiolucent area with
sclerotic, fairly circumscribed upper
border, mimicking a benign lesion.
but the lateral tibial border shows
that the cortex is interrupted and
there is a small interrupted
periosteal reaction
OTHER INVESTIGATIONS
 Routine Blood Tests
 X-RAY CHEST
 MRI
 CT
 Angiogram
 Bone scan
 Biopsy
62
LABORATORY STUDIES
 Full blood count, ESR, CRP.
 LDH (elevated level is associated with
poor prognosis)
 ALP (highly osteogenic)
 Platelet count
 Electrolyte levels
 Liver function tests
 Renal function tests
 Urinalysis
63
MRI
best to detect extent into bone and soft tissues
64
CT
 Not of much use
 CECT chest to detect lung metastasis
65
ANGIOGRAM • Determine vascularity of the tumour
• Detect vascular displacement
• Relationship of vessels to the tumour
66
BONE SCAN
A bone scan should be obtained
to look for skeletal metastases
or multi focal disease
Thallium scan –
Monitor effects of chemotherapy
Detect local recurrence of tumor
67
BIOPSY
 to conform the diagnosis.
 Types
 Fine needle aspiration
 Core needle biopsy
 Open incisional biopsy
68
ENNEKING STAGING SYSTEM
The staging system is typically depicted as follows
Stage I: Low grade tumors
I-A intra compartmental
I-B extra compartmental
Stage II: High grade tumors
II-A intra compartmental
II-B extra compartmental
Stage III: Any tumors with evidence of
metastasis 69
? HOW WE DEFINE AN ANATOMICAL COMPARTMENT
 Anatomical compartment are determined by natural anatomical barriers
to tumor growth, such as cortical bone, articular cartilage, fascial septa, or
joint capsule.
 Stage 1B and 2B lesions extend beyond the compartment of origin.
DIFFERENTIAL DX
 Giant Cell Tumor
 Chondrosarcoma
 Fibrosarcoma
 Aneursymal Bone Cyst
 Ewings sarcoma
 Osteoblastoma
 Metastasis
 Lymphoma
 Osteomyelitis
 Chondroblastoma
 Post traumatic callus
72
PROGNOSTIC FACTORS
 Extent of the disease
 Pts with pulmonary, non pulmonry (bone) or skip metastasis
have poor prognosis
 Grade of the tumor
 High grade tumor have poor prognosis
 Size of the primary lesion
 Large size tumors have worse prognosis then small size tumors
 Skeletal location
 proximal tumors do worse than distal tumors.
 Secondary osteosarcoma: Poor prognosis
73
TREATMENT
 Current standard of care
Radiological staging
Biopsy to confirm diagnosis
Preoperative chemotherapy
Repeat radiological staging (access chemo response,
finalize surgical treatment plan)
Surgical resection with wide margin
Reconstruction using one of many
techniques
Post op chemo based on preop response
74
CHEMOTHERAPY
 Chemotherapy given preoperatively -Neoadjuvant
 Given postoperatively - Adjuvant
 Advantages of neoadjuvant chemotherapy -
 regression of the primary tumor, making a successful limb
salvage operation easier.
 may decrease the spread of tumor cells at the time of
surgery
 Effectively treating micrometastases at the earliest time
possible.
 It avoid tumor progression, which may occur during any
delay before surgery.
 Given for about 3-4 weeks before definitive procedure
75
CHEMOTHERAPY
The drugs used most often to treat
osteosarcoma are:
Methotrexate with leucovorin (folinic
acid)
Doxorubicin (Adriamycin)
Cisplatin or carboplatin
Etoposide
Ifosfamide
Cyclophosphamide
Actinomycin D (dactinomycin)
Bleomycin
76
SURGERY
The main goal of surgery is to safely and
completely remove the tumor.
Historically – amputation.
Over the past few years - limb-sparing
procedures have become the standard,
mainly due to advances in chemotherapy
and sophisticated imaging techniques
Limb salvage procedures now can provide
rates of local control and long-term survival
equal to amputation. 77
SURGERY CHOICE
 Surgical procedures fall into three basic
categories:
 Amputation
 Limb salvage
78
Rotationplasy
DECISION ???
 If the tumor can be removed safely while
retaining a viable extremity, a limb sparing
procedure may be appropriate.
 If major nerves or blood vessels are involved,
or if complete tumor removal results in
significant loss of function, amputation may be
a better choice.
 Patient’s age, desired level of function,
cosmetic preference and long-term prognosis
must also be considered.
79
AMPUTATION
Amputation involves removal of the limb with
a safe margin between the end of the
retained portion and the tumor
It should not be viewed as a failure of
treatment, but rather as the first step towards
patient’s return to a more comfortable and
productive life.
80
AMPUTATION
Indication
1. Grossly displaced pathologic fracture
2. Encasement of neurovascular bundle
3. Tumor that enlarges during preop chemo and is
adjacent to neurovascular bundle
4. Palliative measure in metastatic disease
5. If the tumor has caused massive necrosis,
fungation, infection, or vascular compromise.
81
LIMB SALVAGE SURGERY
Removing the tumor with a normal cuff
of tissue surrounding it while preserving
vascular and nerve supply to the
extremity.
82
 The skeletal defect must be reconstructed by
 Endoprosthesis (most common) –
replacing the removed bone with a metal
implant
 Allograft (cadaveric) bone
 Vascularized bone acquired from the
patient
Allograft-prosthetic composite constructions
83
ROTATIONPLASTY
compromise between amputation and limb
salvage
most commonly used for osteosarcomas of
the distal femur in skeletally immature
patients
It is a procedure where the neurovascular
structures and distal aspect of the limb (leg)
are retained, and re-attached to the
proximal portion after the tumor has been
removed.
84
FOR FUNCTIONAL PURPOSES, THE DISTAL SEGMENT IS TURNED 180
DEGREES SO THAT THE ANKLE JOINT FUNCTIONS AS A KNEE JOINT,
THUS CONVERTING AN ABOVE-KNEE TO A BELOW-KNEE
AMPUTATION IN ORDER FOR PROSTHETIC USE TO BE MAXIMIZED
85
RADIOTHERAPY
 Radiation therapy has no major role in
osteosarcoma
 Radiation therapy may be useful in some cases
where the tumor cannot be completely removed
by surgery. E.g. in pelvic bones or in the bones of
the face.
 Megavoltage (upto 6000-8000 rads)
86
FOLLOW UP AND PROGNOSIS
Signs of recurrence, metastasis and
treatment related complications
Physical examination,radiographs of the
primary site, serial chest imaging,bone scans
and laboratory examinations
50 % cases with high grade osteosarcoma
have some type of relapse in 5 months
If recurrence is detected, additional surgery
(radical amputation)and chemotherapy
may be warranted.
5 year survival rate is 5% - 23% 87
IN SUMMARY
 OS occurs in metaphysis of long bones, slightly more common in males.
 Xrays are must in any pt of 2nd decade complaining of bony pain
 Carefully look for periosteal reaction on xray
 Always get histopathological examination from an expert pathologist
 CECT can help to detect early metastasis
 Prognosis is poor
 Treated with combination of chemotherapy and surgery.
osteosarcoma

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osteosarcoma

  • 2. DEFINITION  It is a malignant mesenchymal tumor in which the tumor cells produce bone matrix or osteoid.
  • 3. OSTEOSARCOMA  It is m/c non-hematologic primary malignancy of bone  the second most common primary malignancy of bone behind multiple myeloma  m/c radiation associated sarcoma  Lungs are m/c site of metastases in OS  .
  • 4. EPIDEMIOLOGY  incidence is 2 cases per million persons per year  peak incidence is in 2nd – 3rd decades.  It is more frequent in tall people than in short people.  Bones having the fastest rates of growth have the highest frequency of occurrence  Slightly more common in males
  • 5. Associated syndromes o Hereditary form of retinoblastoma o Li-Fraumeni syndrome (p53) o Rothmund-Thomson syndrome (8q24) o Beckman-Wiederman syndrome o Paget disease or fibrous dysplasia 5
  • 6. EXCITING FACTORS Some virus like polyoma virus Radiation >2000 rads Chemicals – chlorantherene, Beryllium compounds 6
  • 7.  Arise from multipotent mesenchymal cells  usually in the medullary cavity of the metaphysis of a growing long tubular bone, it may also arise on the surface of a bone, or from an extra-skeletal site.  the majority of medullary origin are high grade, whereas surfaces OS are of lower grade. Origin
  • 8. PATHOGENESIS The tumor arise in the metaphysis where the growth is more active. The tumor originate beneath the periosteum After originating tumor may extend in two directions 1 towards medulla or 2. towards sub- periosteal area
  • 9. This triangular area is known as codmans triangle. In the medulla the bone trabeculae are destroyed Tumor appear as irregular mass permeating along medullary canal At first the periosteum offers impenetrable barrier and is only raised off the bone. On diaphyseal side of tumor the periosteum is often stripped for a short distance There may be deposition of layers of new bone parallel to shaft
  • 10. GROSS PATHOLOGY  OS are bulky tumors that are gritty,  grayish-white in color  areas of hemorrhage and cystic denegation  Destruction of cortex  spread in medullary canal, infiltrating and replacing marrow  Rarely , they penetrate epiphyseal plate or enter the joint  There is Reactive periosteal new bone formation 10
  • 12. MICROSCOPICALLY High grade spindle cell tumor that produces osteoid matrix unconnected by cartilage Tumor cells produce neoplastic bone - basophilic thin trabeculae resembling fungal hyphae or neoplastic osteoid - eosinophilic, homogenous, glassy with irregular contours and osteoblastic rimming Vascular invasion and areas of necrosis common
  • 13. MICROSCOPICALLY May have osteoblastic, fibroblastic or chondroblastic predominance Osteoid in variable amount stroma contain bizarre giant cells or acellular Tumor cells may be spindle, oval or round of variable size 25% of os have osteoclast-like multinucleated giant cells Cartilage may be mineralized, immature, myxoid
  • 14. TYPES OF OSTEOSARCOMA 1. Central a. High-grade  Conventional  Telangiectatic  Small cell  Epithelioid  Osteoblastoma-like  Chondroblastoma-like  Fibrohistiocytic  Giant cell–rich b. Low-grade  Low-grade central  Fibrous dysplasia–like 2. Surface a. Low-grade  Parosteal b. Intermediate-grade  Periosteal c. High-grade  Dedifferentiated parosteal  High-grade surface 3. Intracortical 4. Gnathic 5. Extraskeletal  High-grade  Low-grade
  • 15. CONVENTIONAL OSTEOSARCOMA The tumor cells vary from spindle to polyhedral; their nuclei are pleomorphic and hyperchromatic. Mitotic figures are easily demonstrable. extracellular matrix that may be osseous, cartilaginous, or fibrous in various proportions. The production of bone directly by tumor cells at least somewhere in the tumor is the absolute requirement for diagnosis high-grade tumors and need chemotherapy abundant production of osteoid and bone matrix in a lacy pattern into which the malignant cells are incorporated.
  • 16. TELANGIECTATIC OSTEOSARCOMA  It resembles aneurysmal bone cyst radiographically and histologically.  there is asymmetric expansion of the involved bone with radiolucent bone destruction  but in ABC, a thin shell of periosteal neocortex IS not seen.
  • 17. telangiectatic OS ABC an enhancing thin peripheral rim and septa without nodularity or osteoid matrix mineralization. the growth is less aggressive, with expansile well-defined encapsulated margin, asymmetric expansion, geographic lysis of bone, and an aggressive growth pattern with cortical destruction and minimal peripheral sclerosis and infiltrative margins
  • 18. An eccentric radiolucent destructive lesion. Curetting was done & histopathology showed features of ABC. but One month later, the patient returned with pain and increasing size of mass with destruction and expansion of the entire metaphyseal region of the right humerus. Retrospectively, first radiograph showed a medial interrupted periosteal reaction
  • 19.  At low power, telangiectatic os is composed of multiple blood-filled sinusoids with little solid like aneurysmal bone cyst,  At higher power, there is nuclear pleomorphism and a high mitotic rate.  there is local destruction with permeation to adjacent marrow highly atypical cells lining the septa in a telangiectatic osteosarcoma not seen in ABC presence of several benign giant cells in the septa can be mistaken for giant cell tumor.
  • 20. SMALL CELL OSTEOSARCOMA  a rare histologic variant of osteosarcoma with histologic features combining those of osteosarcoma and Ewing sarcoma.  It constitutes 1% and 2% of all osteosarcomas.  The radiologic features are not typical
  • 21. permeative lytic bone destruction , a soft-tissue mass, and periosteal reaction sometimes with pathological fracture
  • 22.  Histologically, it may be mistaken for Ewing sarcoma/primitive neuroectodermal tumor (PNET) because its cells are small and have round, hyperchromatic nuclei with very little nuclear pleomorphism. the production of osteoid by tumor cells is not a feature of Ewing sarcoma, The tumor above the bone trabecula is composed of small cells dominated by round, blue nuclei suggestive of Ewing sarcoma/primitive neuroectodermal tumor. The tumor below the trabecula demonstrates that the tumor cells are separated by pink osteoid matrix
  • 23. AT HIGHER MAGNIFICATION the tumor cells above the trabecula are indistinguishable from Ewing sarcoma the tumor cells below the trabecula are clearly producing osteoid matrix
  • 24. EPITHELIOID OSTEOSARCOMA  an osteosarcoma in which the tumor cells are so poorly differentiated that it is difficult to determine histologically whether the tumor is a sarcoma or a carcinoma.
  • 25.  The tumor cells often are round or polyhedral rather than spindle- shaped.  Their round or ovoid enlarged nuclei may contain 1 or more prominent nucleoli,  The cells may demonstrate gland-like structures or even contain cells arranged in papillary configurations clusters of polyhedral cells aggregated about central cores resembling papillary structures; some of these contain osteoid rather than vessels.
  • 26. Higher magnification -- epithelioid round cells loosely disposed among a few wisps of osteoid
  • 27.  If tumors like these are seen in young patients, osteosarcoma should be considered.  In older patients, the diagnosis of a poorly differentiated metastatic carcinoma is more likely.
  • 28. OSTEOBLASTOMA-LIKE AND CHONDROBLASTOMA-LIKE OSTEOSARCOMA  Osteoblastoma-like osteosarcoma produces the same sort of microtrabecular bone lined by osteoblasts as does osteoblastoma.  The radiographic appearance often suggests a malignant. a well-circumscribed tumor.
  • 29.  parameters for malignancy include permeation of normal bone at its interface with tumor and aneuploid mitotic activity are seen in os  whereas benign osteoblastic tumors shows maturation and circumscription at their edges. Microtrabecular osteoid lined by osteoblast- like cells as seen in osteoblastomas
  • 30. Chondroblastoma-like osteosarcoma may be epiphyseal. When it is epiphyseal and well circumscribed, it is a very difficult diagnosis to make because radiologic studies suggest a benign diagnosis. In these cases, the diagnosis must be made histologically radiographs demonstrate a well circumscribed radiolucency centered in the distal end of femur, CT- Showed that the lesion is confined to the bone
  • 31. This type of osteosarcoma is best distinguished by its osteoid or bone formation, atypical mitotic activity, and infiltration of adjacent inter-trabecular spaces 1. a monotonous lesion composed of uniform cells with demarcated cytoplasmic borders and with bean-shaped, ovoid nuclei and fine, lattice-like calcifications in a chondroid background 2. more thorough sampling revealed foci of typical lace-like osteoid infiltrating cancellous bone at the periphery, diagnostic of osteosarcoma.
  • 32. GIANT CELL–RICH OSTEOSARCOMA  About 25% of osteosarcomas contain benign multinucleated giant cells.  Rarely, an osteosarcoma may contain so many benign giant cells that the malignant elements in the background are obscured; and the lesion may be mistaken for a giant cell tumor.  This is more apt to occur in the sacrum, where giant cell tumor is much more common radiographs reveal a locally destructive tumor extending from the metaphysis to the articular end of the bone. There is internal circumscription and no periosteal reaction.
  • 33. 1 3 2 2. nuclear atypia and atypical mitotic activity are clearly shown , 1. a giant cell rich lesion with mononuclear background; 3. Individually malignant mononuclear cells and even polyploid giant cells within a collagenized matrix focally having the properties of osteoid
  • 34.  the usual giant cell tumor tends to arise in skeletally mature people and extends from the metaphyseal side of the growth plate to the ends of the long bones.  In the absence of a fracture, a periosteal reaction also is uncommon in an uncomplicated giant cell tumor.  A tumor that appears to be a giant cell tumor histologically but arises in a skeletally immature person, should be regarded with great suspicion and sampled widely to rule out osteosarcoma. Specially if it is accompanied by a visible periosteal reaction.
  • 35. GNATHIC OSTEOSARCOMA Osteosarcomas of the mandible and maxilla usually manifest with swelling or pain. Radiographically, they often are radiolucent or a mixture of radiolucent and radiodense areas.
  • 36.  often are high-grade tumors histologically,  they give rise to uncontrolled local disease,  small minority of patients develope distant metastatic disease Histologic sections demonstrate a mostly cartilaginous, highly cellular tumor that contains foci of immature osteoid production.
  • 37. Low-Grade Central Osteosarcoma unusual variant of osteosarcoma Its microtrabecular osseous matrix architecture in a bland fibrous stroma bears some resemblance to fibrous dysplasia and other benign lesions.  but  atypical mitotic activity may lead to the correct diagnosis. 1.The majority shows immature, poorly formed, woven trabeculae without appositional osteoblastic activity in a modestly cellular fibrous as in fibrous dysplasia but 2. nuclei at higher magnification revealed mitotic activity and atypical mitoses
  • 38.  The imaging features suggests a lesion of greater local aggressiveness than fibrous dysplasia or other benign lesions.  These features include indistinct circumscription, dense sclerosis, an interrupted periosteal reaction, or cortical infraction.  Although patients usually are treated with surgery alone and their prognosis is significantly better than in conventional OS radiographs demonstrate a mottled, circumscribed lesion that resembles fibrous dysplasia except for the very focal area of cortical erosion of the lateral femoral metadiaphysis
  • 39. Surface Osteosarcomas  these are osteosarcomas whose epicenters are outside the cortex of the underlying bone.  They usually arise in relation to the periosteum or the cortex of the bone with minimal or no involvement of the medullary cavity.  usually arises in the third and fourth decade.  The majority are low-grade neoplasms with a propensity for local recurrence and very limited capacity for distant metastasis.  approximately 10% of surface osteosarcomas are high-grade tumors.
  • 40. Parosteal Osteosarcoma  This lesion is often confused with osteochondroma and osteoma  3rd -4th decade  It has a unique presentation and anatomic distribution  75% to 80% of cases arise as densely radiopaque masses attached to the distal posterior femur.  Clinically may give rise to a sensation of fullness, prevent complete range of flexion, and perceived as a mass through the overlying soft tissues,  it is unusual for the entity to be spontaneously painful.
  • 41.  Its radiodensity often is not uniform, often being more radiodense centrally and less dense at its periphery.  In contrast with osteochondroma, there is no usual continuity with the adjacent cortex, and its inside is not in continuity with the underlying medullary cavity  In localized heterotopic ossification (so-called myositis ossificans ) the ossification pattern is exactly the opposite radiographically, with the densest bone being outside and the least dense being inside.  In parosteal osteosarcoma, there is an incomplete radiolucent line separating most of the sclerotic mass from the underlying cortex,  this radiolucent line corresponds to the underling periosteum, to which the mass usually is fixed at one point on the outside.  An underlying periosteal reaction is very unusual because to produce such a reaction, the tumor would have to lift the inner cambium layer of the periosteum away from the cortex.  Parosteal osteosarcoma usually is not locally aggressive enough to do this. sclerotic mass, partially ill-defined posteriorly to the distal femur
  • 42.  Histologically, parosteal osteosarcomas consist of streamers of bone trabeculae that often show a high degree of parallel orientation similar to that observed in a periosteal new bone reaction.  There is histologic resemblance of this bone formation to “flowing steel wool.”  There is a cellular cartilage cap external to the osseous portion of the lesion in about 25% to 30% of cases. flowing steel wool appearance cartilage cap
  • 43.  The histologic key to the correct diagnosis is that a growing osteochondroma has the same internal development as a growing bone.  There is a medullary cavity with fat or hematopoietic marrow in the intertrabecular spaces of normal bone and osteochondroma, whereas the intertrabecular spaces of parosteal osteosarcomas contain cellular fibrous tissue.  The majority of parosteal osteosarcomas are associated with the outer fibrous layer of the periosteum, which does not produce typical periosteal reactions. Although the fibrous tissue between the bone spicules is bland, there is no marrow evident. A careful search in the fibrous component sometimes reveals focal hypercellularity and early immature bone formation
  • 44. Periosteal Osteosarcoma  Although the lesion is associated with the bone surface, it arises between the cortex and the cambium layer of the periosteum, so that there often is a periosteal reaction visible radiographically.  There is underlying cortical thickening or erosion  it usually occurs along the tibial or femoral diaphysis rather than posterior to the metaphysis of the distal femur.  Its histologic grade is intermediate  rarely it may extend into the underlying cortex, but never is extend into the underlying endosteum
  • 45. Dedifferentiated Parosteal Osteosarcoma  histologically high-grade  arising in the clinical setting of recurrent parosteal osteosarcoma of the usual low-grade variety.  the dedifferentiation took place over time in recurrences of well- documented tumors that previously were diagnosed as typical low-grade parosteal osteosarcomas. cloudy, radiodense tumor matrix in the soft tissues. the portion of lateral femoral condyle that does not overlap the patella and the upper medial metaphysis are markedly radiolucent compared with the bone of the metadiaphysis
  • 46.  Histologically, the tissue is composed of an admixture of typical appearing low-grade parosteal osteosarcoma and high-grade conventional parosteal osteosarcoma  The is worse than that of low-grade parosteal osteosarcoma but somewhat better than that of pure high-grade surface
  • 47. HIGH-GRADE SURFACE OSTEOSARCOMA  It manifests as a surface lesion of bone, but is high grade histologically.  It may appear radiographically as an ordinary low-grade parosteal osteosarcoma with dense sclerosis.  its local growth and aggressiveness are more accelerated.  patients with this disease have more distressing symptoms and signs than those with low-grade parosteal osteosarcomas.  the underlying tibia shows no gross invasion by the tumor
  • 48.  Microscopically, the tumor is entirely high grade.  There usually is very little or no medullary invasion by the tumor on xrays. a high-grade neoplasm producing wispy osteoid A computed tomography scan shows separation of the mass from the underlying bone by a dark periosteal line.
  • 49. Intracortical Osteosarcoma  very rare anatomic variant of osteosarcoma,  a high-grade osteosarcoma entirely confined within the bony cortex.  manifests as an area of cortical radiolucency with peri-lesional sclerosis.  Depending on its size, it may be confused with osteoid osteoma and osteoblastoma, but it has cellular atypia and is locally aggressive  Histologically, there is abundant osteoid of bone formation.
  • 50. MULTIFOCAL OSTEOSARCOMA  This extremely unusual condition affects multiple osseous sites simultaneously at the time of presentation (synchronous form) or  multiple skeletal sites at varying intervals (metachronous type) .  The former variant tends to affect children and adolescents, whereas the latter type occurs in adults.  The childhood synchronous form is radiodense, usually symmetric, and a high-grade sarcoma with a rapidly fatal course.  In the adult metachronous form, after the appearance of the first osteosarcoma, there is a disease-free interval of several months to years followed by the development of one or several skeletal osteosarcomas.  These lesions are not symmetric, they may have variable degrees of sclerosis, and their histologic features may vary from high to low grade.  The metachronous form have a longer survival interval than children with the synchronous form Synchronous Multifocal Osteosarcoma
  • 51. EXTRA-SKELETAL OSTEOSARCOMA  These accounts for less than 2% of all soft tissue sarcomas.  it tends to occur in late adulthood,  the fifth to seventh decades of life  Most cases arise in the deep soft tissues with a predilection for the thigh followed by the buttocks, upper extremities, and the retroperitoneum.
  • 52.  Patients usually have an enlarging soft tissue mass, and the mass may be painful  Radiographs show a mass with mineralization  Histologically, the tumors demonstrate all types of osteosarcoma seen in bone tumors.  The prognosis is poor in soft tissue osteosarcoma of the high- grade type.  patients with the low-grade variant seem to have a better prognosis
  • 53. SKELETAL DISTRIBUTION  Distal femur  Proximal tibia  Proximal humerus (sites of rapid bone growth)  others Metaphyseal(89%)>diaphyseal(10%)>epiphyseal(1%) 53
  • 54. CLINICAL PRESENTATION  m/c symptom -pain for several weeks or months.  The most common sign is a mass of variable consistency( soft to hard) and tender.  There may be superficial erythema, venous distension, or other signs of hyper-vascularity.
  • 55. There may be a limp, loss of function, or even decreased range of motion Fever, malaise or other constitutional symptoms are not typical of osteosarcoma
  • 56. COURSE  Metastasis occur early by hematogenous rout  Progressive pulmonary metastasis develop very early  Process is usually fatal within 2 years
  • 57. X- RAY  Variable portions of radiopacities and radiolucencies,  medullary and cortical bone destruction  wide zone of transition, permeative or moth-eaten appearance  aggressive periosteal reaction  sunburst type  Codman triangle  lamellated (onion skin) reaction: less frequently seen  soft-tissue mass  tumour matrix ossification/calcification
  • 58.  The cambium layer of the inner periosteum reacts to separation from the cortex by producing new bone, which is sometimes visible as an incomplete bony shell that appears attached to the bone surface on only one end and is open in the middle, so-called Codman angle.  Although periosteal new bone formation sometimes is associated with benign conditions, periosteal reactions associated with malignant tumors usually are discontinuous, implying that the tumor growth is too rapid for periosteal containment.
  • 59.  Lesions are usually permeative  Associated with destruction of the cancellous and cortical elements of the bone  Ossification within the soft tissue component, if tumour has broken through cortex  Borders are ill defined Plain X-ray 59
  • 60. PLAIN X-RAY Periosteal reaction may appear as the characteristic Codman triangle. Extension of the tumor through the periosteum may result in a so-called “sunburst” or “hair on end” appearance.
  • 61. QUESTION A completely radiolucent area with sclerotic, fairly circumscribed upper border, mimicking a benign lesion. but the lateral tibial border shows that the cortex is interrupted and there is a small interrupted periosteal reaction
  • 62. OTHER INVESTIGATIONS  Routine Blood Tests  X-RAY CHEST  MRI  CT  Angiogram  Bone scan  Biopsy 62
  • 63. LABORATORY STUDIES  Full blood count, ESR, CRP.  LDH (elevated level is associated with poor prognosis)  ALP (highly osteogenic)  Platelet count  Electrolyte levels  Liver function tests  Renal function tests  Urinalysis 63
  • 64. MRI best to detect extent into bone and soft tissues 64
  • 65. CT  Not of much use  CECT chest to detect lung metastasis 65
  • 66. ANGIOGRAM • Determine vascularity of the tumour • Detect vascular displacement • Relationship of vessels to the tumour 66
  • 67. BONE SCAN A bone scan should be obtained to look for skeletal metastases or multi focal disease Thallium scan – Monitor effects of chemotherapy Detect local recurrence of tumor 67
  • 68. BIOPSY  to conform the diagnosis.  Types  Fine needle aspiration  Core needle biopsy  Open incisional biopsy 68
  • 69. ENNEKING STAGING SYSTEM The staging system is typically depicted as follows Stage I: Low grade tumors I-A intra compartmental I-B extra compartmental Stage II: High grade tumors II-A intra compartmental II-B extra compartmental Stage III: Any tumors with evidence of metastasis 69
  • 70. ? HOW WE DEFINE AN ANATOMICAL COMPARTMENT  Anatomical compartment are determined by natural anatomical barriers to tumor growth, such as cortical bone, articular cartilage, fascial septa, or joint capsule.  Stage 1B and 2B lesions extend beyond the compartment of origin.
  • 71.
  • 72. DIFFERENTIAL DX  Giant Cell Tumor  Chondrosarcoma  Fibrosarcoma  Aneursymal Bone Cyst  Ewings sarcoma  Osteoblastoma  Metastasis  Lymphoma  Osteomyelitis  Chondroblastoma  Post traumatic callus 72
  • 73. PROGNOSTIC FACTORS  Extent of the disease  Pts with pulmonary, non pulmonry (bone) or skip metastasis have poor prognosis  Grade of the tumor  High grade tumor have poor prognosis  Size of the primary lesion  Large size tumors have worse prognosis then small size tumors  Skeletal location  proximal tumors do worse than distal tumors.  Secondary osteosarcoma: Poor prognosis 73
  • 74. TREATMENT  Current standard of care Radiological staging Biopsy to confirm diagnosis Preoperative chemotherapy Repeat radiological staging (access chemo response, finalize surgical treatment plan) Surgical resection with wide margin Reconstruction using one of many techniques Post op chemo based on preop response 74
  • 75. CHEMOTHERAPY  Chemotherapy given preoperatively -Neoadjuvant  Given postoperatively - Adjuvant  Advantages of neoadjuvant chemotherapy -  regression of the primary tumor, making a successful limb salvage operation easier.  may decrease the spread of tumor cells at the time of surgery  Effectively treating micrometastases at the earliest time possible.  It avoid tumor progression, which may occur during any delay before surgery.  Given for about 3-4 weeks before definitive procedure 75
  • 76. CHEMOTHERAPY The drugs used most often to treat osteosarcoma are: Methotrexate with leucovorin (folinic acid) Doxorubicin (Adriamycin) Cisplatin or carboplatin Etoposide Ifosfamide Cyclophosphamide Actinomycin D (dactinomycin) Bleomycin 76
  • 77. SURGERY The main goal of surgery is to safely and completely remove the tumor. Historically – amputation. Over the past few years - limb-sparing procedures have become the standard, mainly due to advances in chemotherapy and sophisticated imaging techniques Limb salvage procedures now can provide rates of local control and long-term survival equal to amputation. 77
  • 78. SURGERY CHOICE  Surgical procedures fall into three basic categories:  Amputation  Limb salvage 78 Rotationplasy
  • 79. DECISION ???  If the tumor can be removed safely while retaining a viable extremity, a limb sparing procedure may be appropriate.  If major nerves or blood vessels are involved, or if complete tumor removal results in significant loss of function, amputation may be a better choice.  Patient’s age, desired level of function, cosmetic preference and long-term prognosis must also be considered. 79
  • 80. AMPUTATION Amputation involves removal of the limb with a safe margin between the end of the retained portion and the tumor It should not be viewed as a failure of treatment, but rather as the first step towards patient’s return to a more comfortable and productive life. 80
  • 81. AMPUTATION Indication 1. Grossly displaced pathologic fracture 2. Encasement of neurovascular bundle 3. Tumor that enlarges during preop chemo and is adjacent to neurovascular bundle 4. Palliative measure in metastatic disease 5. If the tumor has caused massive necrosis, fungation, infection, or vascular compromise. 81
  • 82. LIMB SALVAGE SURGERY Removing the tumor with a normal cuff of tissue surrounding it while preserving vascular and nerve supply to the extremity. 82
  • 83.  The skeletal defect must be reconstructed by  Endoprosthesis (most common) – replacing the removed bone with a metal implant  Allograft (cadaveric) bone  Vascularized bone acquired from the patient Allograft-prosthetic composite constructions 83
  • 84. ROTATIONPLASTY compromise between amputation and limb salvage most commonly used for osteosarcomas of the distal femur in skeletally immature patients It is a procedure where the neurovascular structures and distal aspect of the limb (leg) are retained, and re-attached to the proximal portion after the tumor has been removed. 84
  • 85. FOR FUNCTIONAL PURPOSES, THE DISTAL SEGMENT IS TURNED 180 DEGREES SO THAT THE ANKLE JOINT FUNCTIONS AS A KNEE JOINT, THUS CONVERTING AN ABOVE-KNEE TO A BELOW-KNEE AMPUTATION IN ORDER FOR PROSTHETIC USE TO BE MAXIMIZED 85
  • 86. RADIOTHERAPY  Radiation therapy has no major role in osteosarcoma  Radiation therapy may be useful in some cases where the tumor cannot be completely removed by surgery. E.g. in pelvic bones or in the bones of the face.  Megavoltage (upto 6000-8000 rads) 86
  • 87. FOLLOW UP AND PROGNOSIS Signs of recurrence, metastasis and treatment related complications Physical examination,radiographs of the primary site, serial chest imaging,bone scans and laboratory examinations 50 % cases with high grade osteosarcoma have some type of relapse in 5 months If recurrence is detected, additional surgery (radical amputation)and chemotherapy may be warranted. 5 year survival rate is 5% - 23% 87
  • 88. IN SUMMARY  OS occurs in metaphysis of long bones, slightly more common in males.  Xrays are must in any pt of 2nd decade complaining of bony pain  Carefully look for periosteal reaction on xray  Always get histopathological examination from an expert pathologist  CECT can help to detect early metastasis  Prognosis is poor  Treated with combination of chemotherapy and surgery.