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Presented By
Dr. Santosh Atreya
MD Resident,Phase-A,BSMMU
Outline of Presentation
What is Osteosarcoma?
Characteristics
Gross Pathology and Appearance
Classification
Clinical Presentation
Regional Distribution
Diagnosis/Radiological
Shortly about Parosteal, Periostial osteosarcoma
Treatment and Prognosis
D/D of Osteosarcoma
Difference among Osteosarcoma, Osteomyelitis and
Ewing Sarcoma
Osteo=bone/osteoid tissue.
Sarcoma=malignant tumour of
connective tissue.
An osteosarcoma is the commonest
primary malignant bone tumour.
They account for 25 % of all primary bone
tumors.
Age: 10-25 years
In older age groups associated with Pagets
disease
Gender: slight male preponderance exists
Incidence
Location
Any bone may be involved, rather
more than half located around the
knee involving the metadiaphyses
of the distal end of femur and
proximal end of tibia
Infrequently in pelvis, spine
Clavicle ribs scapula and small
bones of hands and feet -rare
10% arise in diaphysis
Clinical Presentation
Patients usually present
with localised pain or
swelling particularly around
the knee, occasionally
accompanied by a soft-
tissue mass or swelling.
Sometimes, the first
symptoms are related to
pathological fracture.
Characteristics
It is histologically pleomorphic.
Two diagnostic features are –
a)It’s ability to produce osteoid tissue without
necessarily the development of cartilaginous
precursor.
b)The presence of abundant alkaline phosphatase
histochemically within the tumor cells
Pleomorphic nature of sarcoma
A dominant cell line may modify the
appearance.
If osteoblasts predominate, tumour bone
formation will result.
Whereas if cells of cartilage origin are
present, extensive calcification may be a
presenting feature.
Fig:1.Osteosarcoma of the tibia and
fibula-predominantly
osteoblastic.Amorphous
calcification/ossification is present
in the soft tissues with cortical
destruction and a little periosteal
new bone formation.
Fig:2.Osteosarcoma of the distal
femur-predominantly
chondroblastic.Note the well-
defined soft tissue mass and
radiating spiculation of
calcification within it.
Metastasis: It is highly vascular & metastases
occurs by hematogenous route to the lung.
Any lung lesion arising in a patient with
osteosarcoma should be regarded with
suspicion. .Later stage metastasis may spread
to bone.
Skip metastases-5 to 8%. Lymphatic spread
is rare.
Fig: Osteosarcoma-metastasis in the lungs
presents with a pneumothorax.
Gross pathology
Arise from multipotent
mesenchymal cells.
Mixture of osteoid, fibrous,
cartilaginous tissue,necrotic,
hemorrhagic,cystic areas,
destruction of cortex
Arise eccentrically in the
medullary cavity with ill
defined cortical destruction
and soft tissue involvement.
Gross Appearance
Large tumors
Gritty & grayish-white in color
Hemorrhage
Cystic degeneration
Cortical destruction
Spreads – medullary canal
Soft tissue masses present
Classification
A.Primary osteosarcoma
B.Secondary osteosarcoma
A.Primary osteosarcoma:
According to dominant cell line( Histopathology) classified as-
a. Osteoblastic
b. Chondroblastic
c. Fibroblastic
d. Anaplastic and e.Telangiectatic
Accoarding to site:
a.Diaphyseal
b.Central
c.Multifocal and
d.Soft tissue osteosarcoma
B.Secondary osteosarcoma:
Secondary to-
I. Paget’s disease(paget’s sarcoma)
II. Radiation or ingestion of radioactive material.
S.N PRIMARY OSTEOSARCOMA SECONDARY OSTEOSARCOMA
1. In young patients (10 - 25 years) Occurs in the elderly
2. 75% < age of 20 Secondary to malignant
degeneration of pagets
disease,extensive bone infarcts or
post-radiotherapy
3. M>F M>F
4. Typically occur in the metaphyseal
regions of long bones, and have a
striking predilection for the knee
(60%)
Wider distribution,higher incidence
in flat bones, especially the pelvis.
Diagnosis
1.Laboratory studies:
CBC, ESR, CRP, LDH (elevated level indicates
poor prognosis)
ALP (Highly Osteogenic)
Platelet count, Electrolyte levels, Liver function
tests, Renal function tests, Urinalysis
2.Radiological features:
Findings:
Typical appearances include:
Medullary and cortical bone destruction
Wide zone of transition
Permeative or moth-eaten appearance
Aggressive periosteal reaction
Codman triangle
 Sunburst type
 lamellated (onion skin) reaction( less
frequently seen)
soft-tissue mass
tumour matrix ossification / calcification
variable: reflects a combination of the
amount of tumour bone production,
calcified matrix, and osteoid
Cont..
3. CT Scan:
 It is the most sensitive means in detecting
pulmonary metastasis.
CT scanning may be helpful locally when the
radiographic appearances are confusing,
particularly in areas of complex anatomy.
Cross-sectional images provide a clearer
information of bone destruction, as well as
the extent of any soft tissue mass, than do
radiographs.
Cont..
4. MRI
 It is the prime investigation of choice for
Osteosarcoma
 An obvious heterogeneous tumor is
demonstrated with surrounded bones and
usually a soft tissue mass
 Intramedullary skip lesion may also be identified
T1WI
soft tissue non-mineralized component :
intermediate signal intensity
mineralised / ossified components : low signal
intensity
peri-tumoural oedema : intermediate signal
intensity
scattered regions of haemorrhage will have
variable signal
T2WI
soft tissue non-mineralized component : high
signal intensity
mineralised / ossified components : low signal
intensity
peri-tumoural oedema : high signal intensity
abnormal signal intensity
in the metaphyseal
marrow and the soft
tissue mass (black
arrow). Early tumor
extension is shown
beyond the growth
plate into the epiphysis
(white arrows).
Coronal T1-weighted MRI.
STIR suppresses signal from fat,
Sensitive to edema and bone pathology
Normal marrow and fat: dark
Fluid & edema: bright
Bone findings:
1. Increased signal in the medullary
canal.
2. Irregular pattern in the metaphysis.
3. Ill defined cortical outline.
4. Extension to the epiphysis.
5. High signal around distal femur,
suggesting edema and growth into the
surrounding tissue.
Coronal STIR of the left knee
Cont..
5.Biopsy to confirm the diagnosis.
Histology confirmed
radiological suspicion of
osteosarcoma in the
distal femur of patient
1. Formation of new, abnormal
bone with a coarse lacelike
architecture
2. Variable tumor cell size &
shape, with hyperchromatic
nuclei and mitoses.
Cont..
6. Scintigraphy
Osteosarcomas typically
show increased uptake of
radioisotope on bone
scans obtained by use of
technetium-99m (99m
Tc)
methylene
diphosphonate (MDP). A scan in the early blood-pool (left)
and delayed phases (right)
demonstrates an extensive
abnormality. the activity is more
uniform and extensive than the
apparent involvement shown on the
plain film.
Cont..
7. Angiogram
Angiogram Determine vascularity of the tumour ,Detect vascular
displacement and relationship of vessels to the tumour
Telangiectatic osteosarcoma of the
distal femur. predominantly
radiolucent defect is shown on
conventional radiograph.
Angiographically is shown to
contain large, tortuous,
pathological vessels.
Some confusion in nomenclature relates to
osteosarcoma arising in or near the periosteum.
They are divided into two groups:
a.Parosteal osteosarcoma and
b.Periosteal osteosarcoma
Parosteal Osteosarcoma
Most patient affected in 3rd
& 4th
decade.
Typically dense tumour surrounds a long
bone,particularly femur or a tibia. Margin are
sharply defined but tend to undulate.
 The tumour is denser centrally and at the
base than peripherally.
Characteristically there is a radiolucent zone
between the ossified outer margins of the
tumour and adjacent host bone.
Usually, the tumour appears to be attached
to the cortex by a broad pedicle.
Parosteal osteosarcoma of the
proximal humerus. A well-defined
mass of dense tumour bone
surrounds the humeral shaft.
Parosteal osteosarcoma arising from
the anterior aspect of the femur shown
angiographically to be unremarkable
apart from a slight increase in the
number of branches going into the
tumour.
PAROSTEAL OSTEOSARCOMA
Telangiectatic Osteosarcoma (2.5-
12.5%)
Lytic tumors consisting of large cystic cavities
filled with blood usually diametaphyseal in
location.
 Has been considered more aggressive than
classic osteosarcoma, but studies of long-term
survival after optimum treatment now indicate
that the aggressiveness of telangiectatic
osteosarcoma is similar to that of the classic
type.
Frontal radiograph of the distal femur in a patient with
telangiectatic osteosarcoma. the radiograph shows
mixed medullary sclerosis and lucency, cortical
destruction medially, aggressive periosteal changes,
and a large soft-tissue mass with peripheral
ossification
Sarcoma in Paget’s Disease
Malignant tumours are said to arise in bone affected
by Paget's disease in about 1 % of cases.
Overall, the skull, pelvis and long bones are typical
sites, predilection for the humerus in the later.
Men are more commonly affected, even allowing for
the increased male incidence of Paget's disease.
 However, the tumour is very aggressive and the
outlook is very poor.
Radiologically, the lesion is lytic, mixed or
sclerotic.
Xray of the proximal femur in a patient
with Paget disease demonstrates the
typical features of cortical thickening,
osseous expansion, and trabecular
coarsening. In addition, irregular bone
lucency and cortical destruction are
shown in the medial aspect of the shaft;
Coronal T1WIof the same patient
showing -the tumor is shown in
the proximal shaft of the right
femur (white arrow), with cortical
destruction and a large soft-tissue
component (black arrow).
Treatment and Prognosis
Treatment options for classic osteosarcoma
Surgery alone: 20% cure rate.
Surgery & chemotherapy: 60-80% cure rate.
Radical surgical treatment
• Wide surgical resection
• Limb salvage (used in 80-90% of all cases)
Bone replaced with a bone allograft or a
prosthesis.
• Amputation
Currently, the 5-year survival rate after adequate
therapy is approximately 60 - 80% .
Differential Diagnosis
Osteomyelitis
Other tumours :
metastatic lesion to bone
Malignant round cell tumours (Ewing
sarcoma)
Age
Age is the most important clue in differentiating
possible bone tumors.
Osteosarcoma-Between 10 &25 yrs
Ewing’s sarcoma-5 to 30 yrs.
Location within the skeleton
The location of a bone lesion within the skeleton can
be a clue in differential diagnosis.
• Osteomyelitis-femur, tibia, humerus, fibula, radius
• Osteosarcoma-femur
• Ewing's sarcoma-iliac bone, fibula, rib, tibia,
humerus,pelvis.
Site and Location
Osteosarcoma Osteomyelitis Ewing’s
sarcoma
Site Metaphyseal Metaphysis Diaphysis
Location juxtacortical
centric
Eccentric
juxtacortical centric
Periosteal reaction & Zone of
transition
osteosarcoma Osteomyelitis Ewing’s
sarcoma
Periosteal
reaction
Sunburst
spiculation
Formation of
involucrum,se
questra
Onion peel
lameller type
zone of
transition
Wide zone of
transition
Wide zone of
transition
Wide zone of
transition
Ewing's
sarcoma.
well-defined
soft-tissue mass.
Advanced osteomvelitis
involving the whole of
the right tibia and
lowervoend of fibula.
Note sequestrum in tibia
and further sequestrum
being extruded from the
fibula.
Osteosarcoma of the
distal femur-. well-
defined soft-tissue mass
and radiating spiculation
of calcification within it.
Thank You

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Osteosarcoma (knee joint)

  • 1. Presented By Dr. Santosh Atreya MD Resident,Phase-A,BSMMU
  • 2. Outline of Presentation What is Osteosarcoma? Characteristics Gross Pathology and Appearance Classification Clinical Presentation Regional Distribution Diagnosis/Radiological Shortly about Parosteal, Periostial osteosarcoma Treatment and Prognosis D/D of Osteosarcoma Difference among Osteosarcoma, Osteomyelitis and Ewing Sarcoma
  • 3.
  • 4. Osteo=bone/osteoid tissue. Sarcoma=malignant tumour of connective tissue. An osteosarcoma is the commonest primary malignant bone tumour. They account for 25 % of all primary bone tumors.
  • 5. Age: 10-25 years In older age groups associated with Pagets disease Gender: slight male preponderance exists Incidence
  • 6. Location Any bone may be involved, rather more than half located around the knee involving the metadiaphyses of the distal end of femur and proximal end of tibia Infrequently in pelvis, spine Clavicle ribs scapula and small bones of hands and feet -rare 10% arise in diaphysis
  • 7. Clinical Presentation Patients usually present with localised pain or swelling particularly around the knee, occasionally accompanied by a soft- tissue mass or swelling. Sometimes, the first symptoms are related to pathological fracture.
  • 8. Characteristics It is histologically pleomorphic. Two diagnostic features are – a)It’s ability to produce osteoid tissue without necessarily the development of cartilaginous precursor. b)The presence of abundant alkaline phosphatase histochemically within the tumor cells
  • 9. Pleomorphic nature of sarcoma A dominant cell line may modify the appearance. If osteoblasts predominate, tumour bone formation will result. Whereas if cells of cartilage origin are present, extensive calcification may be a presenting feature.
  • 10. Fig:1.Osteosarcoma of the tibia and fibula-predominantly osteoblastic.Amorphous calcification/ossification is present in the soft tissues with cortical destruction and a little periosteal new bone formation. Fig:2.Osteosarcoma of the distal femur-predominantly chondroblastic.Note the well- defined soft tissue mass and radiating spiculation of calcification within it.
  • 11. Metastasis: It is highly vascular & metastases occurs by hematogenous route to the lung. Any lung lesion arising in a patient with osteosarcoma should be regarded with suspicion. .Later stage metastasis may spread to bone. Skip metastases-5 to 8%. Lymphatic spread is rare. Fig: Osteosarcoma-metastasis in the lungs presents with a pneumothorax.
  • 12. Gross pathology Arise from multipotent mesenchymal cells. Mixture of osteoid, fibrous, cartilaginous tissue,necrotic, hemorrhagic,cystic areas, destruction of cortex Arise eccentrically in the medullary cavity with ill defined cortical destruction and soft tissue involvement.
  • 13. Gross Appearance Large tumors Gritty & grayish-white in color Hemorrhage Cystic degeneration Cortical destruction Spreads – medullary canal Soft tissue masses present
  • 14. Classification A.Primary osteosarcoma B.Secondary osteosarcoma A.Primary osteosarcoma: According to dominant cell line( Histopathology) classified as- a. Osteoblastic b. Chondroblastic c. Fibroblastic d. Anaplastic and e.Telangiectatic Accoarding to site: a.Diaphyseal b.Central c.Multifocal and d.Soft tissue osteosarcoma
  • 15. B.Secondary osteosarcoma: Secondary to- I. Paget’s disease(paget’s sarcoma) II. Radiation or ingestion of radioactive material.
  • 16. S.N PRIMARY OSTEOSARCOMA SECONDARY OSTEOSARCOMA 1. In young patients (10 - 25 years) Occurs in the elderly 2. 75% < age of 20 Secondary to malignant degeneration of pagets disease,extensive bone infarcts or post-radiotherapy 3. M>F M>F 4. Typically occur in the metaphyseal regions of long bones, and have a striking predilection for the knee (60%) Wider distribution,higher incidence in flat bones, especially the pelvis.
  • 17. Diagnosis 1.Laboratory studies: CBC, ESR, CRP, LDH (elevated level indicates poor prognosis) ALP (Highly Osteogenic) Platelet count, Electrolyte levels, Liver function tests, Renal function tests, Urinalysis
  • 18. 2.Radiological features: Findings: Typical appearances include: Medullary and cortical bone destruction Wide zone of transition Permeative or moth-eaten appearance Aggressive periosteal reaction Codman triangle  Sunburst type  lamellated (onion skin) reaction( less frequently seen) soft-tissue mass tumour matrix ossification / calcification variable: reflects a combination of the amount of tumour bone production, calcified matrix, and osteoid
  • 19. Cont.. 3. CT Scan:  It is the most sensitive means in detecting pulmonary metastasis. CT scanning may be helpful locally when the radiographic appearances are confusing, particularly in areas of complex anatomy. Cross-sectional images provide a clearer information of bone destruction, as well as the extent of any soft tissue mass, than do radiographs.
  • 20. Cont.. 4. MRI  It is the prime investigation of choice for Osteosarcoma  An obvious heterogeneous tumor is demonstrated with surrounded bones and usually a soft tissue mass  Intramedullary skip lesion may also be identified
  • 21. T1WI soft tissue non-mineralized component : intermediate signal intensity mineralised / ossified components : low signal intensity peri-tumoural oedema : intermediate signal intensity scattered regions of haemorrhage will have variable signal T2WI soft tissue non-mineralized component : high signal intensity mineralised / ossified components : low signal intensity peri-tumoural oedema : high signal intensity
  • 22. abnormal signal intensity in the metaphyseal marrow and the soft tissue mass (black arrow). Early tumor extension is shown beyond the growth plate into the epiphysis (white arrows). Coronal T1-weighted MRI.
  • 23. STIR suppresses signal from fat, Sensitive to edema and bone pathology Normal marrow and fat: dark Fluid & edema: bright Bone findings: 1. Increased signal in the medullary canal. 2. Irregular pattern in the metaphysis. 3. Ill defined cortical outline. 4. Extension to the epiphysis. 5. High signal around distal femur, suggesting edema and growth into the surrounding tissue. Coronal STIR of the left knee
  • 24. Cont.. 5.Biopsy to confirm the diagnosis. Histology confirmed radiological suspicion of osteosarcoma in the distal femur of patient 1. Formation of new, abnormal bone with a coarse lacelike architecture 2. Variable tumor cell size & shape, with hyperchromatic nuclei and mitoses.
  • 25. Cont.. 6. Scintigraphy Osteosarcomas typically show increased uptake of radioisotope on bone scans obtained by use of technetium-99m (99m Tc) methylene diphosphonate (MDP). A scan in the early blood-pool (left) and delayed phases (right) demonstrates an extensive abnormality. the activity is more uniform and extensive than the apparent involvement shown on the plain film.
  • 26. Cont.. 7. Angiogram Angiogram Determine vascularity of the tumour ,Detect vascular displacement and relationship of vessels to the tumour Telangiectatic osteosarcoma of the distal femur. predominantly radiolucent defect is shown on conventional radiograph. Angiographically is shown to contain large, tortuous, pathological vessels.
  • 27. Some confusion in nomenclature relates to osteosarcoma arising in or near the periosteum. They are divided into two groups: a.Parosteal osteosarcoma and b.Periosteal osteosarcoma
  • 28. Parosteal Osteosarcoma Most patient affected in 3rd & 4th decade. Typically dense tumour surrounds a long bone,particularly femur or a tibia. Margin are sharply defined but tend to undulate.  The tumour is denser centrally and at the base than peripherally. Characteristically there is a radiolucent zone between the ossified outer margins of the tumour and adjacent host bone. Usually, the tumour appears to be attached to the cortex by a broad pedicle.
  • 29. Parosteal osteosarcoma of the proximal humerus. A well-defined mass of dense tumour bone surrounds the humeral shaft. Parosteal osteosarcoma arising from the anterior aspect of the femur shown angiographically to be unremarkable apart from a slight increase in the number of branches going into the tumour.
  • 31. Telangiectatic Osteosarcoma (2.5- 12.5%) Lytic tumors consisting of large cystic cavities filled with blood usually diametaphyseal in location.  Has been considered more aggressive than classic osteosarcoma, but studies of long-term survival after optimum treatment now indicate that the aggressiveness of telangiectatic osteosarcoma is similar to that of the classic type.
  • 32. Frontal radiograph of the distal femur in a patient with telangiectatic osteosarcoma. the radiograph shows mixed medullary sclerosis and lucency, cortical destruction medially, aggressive periosteal changes, and a large soft-tissue mass with peripheral ossification
  • 33. Sarcoma in Paget’s Disease Malignant tumours are said to arise in bone affected by Paget's disease in about 1 % of cases. Overall, the skull, pelvis and long bones are typical sites, predilection for the humerus in the later. Men are more commonly affected, even allowing for the increased male incidence of Paget's disease.  However, the tumour is very aggressive and the outlook is very poor. Radiologically, the lesion is lytic, mixed or sclerotic.
  • 34. Xray of the proximal femur in a patient with Paget disease demonstrates the typical features of cortical thickening, osseous expansion, and trabecular coarsening. In addition, irregular bone lucency and cortical destruction are shown in the medial aspect of the shaft; Coronal T1WIof the same patient showing -the tumor is shown in the proximal shaft of the right femur (white arrow), with cortical destruction and a large soft-tissue component (black arrow).
  • 35. Treatment and Prognosis Treatment options for classic osteosarcoma Surgery alone: 20% cure rate. Surgery & chemotherapy: 60-80% cure rate. Radical surgical treatment • Wide surgical resection • Limb salvage (used in 80-90% of all cases) Bone replaced with a bone allograft or a prosthesis. • Amputation Currently, the 5-year survival rate after adequate therapy is approximately 60 - 80% .
  • 36. Differential Diagnosis Osteomyelitis Other tumours : metastatic lesion to bone Malignant round cell tumours (Ewing sarcoma)
  • 37. Age Age is the most important clue in differentiating possible bone tumors. Osteosarcoma-Between 10 &25 yrs Ewing’s sarcoma-5 to 30 yrs. Location within the skeleton The location of a bone lesion within the skeleton can be a clue in differential diagnosis. • Osteomyelitis-femur, tibia, humerus, fibula, radius • Osteosarcoma-femur • Ewing's sarcoma-iliac bone, fibula, rib, tibia, humerus,pelvis.
  • 38. Site and Location Osteosarcoma Osteomyelitis Ewing’s sarcoma Site Metaphyseal Metaphysis Diaphysis Location juxtacortical centric Eccentric juxtacortical centric
  • 39. Periosteal reaction & Zone of transition osteosarcoma Osteomyelitis Ewing’s sarcoma Periosteal reaction Sunburst spiculation Formation of involucrum,se questra Onion peel lameller type zone of transition Wide zone of transition Wide zone of transition Wide zone of transition
  • 40. Ewing's sarcoma. well-defined soft-tissue mass. Advanced osteomvelitis involving the whole of the right tibia and lowervoend of fibula. Note sequestrum in tibia and further sequestrum being extruded from the fibula. Osteosarcoma of the distal femur-. well- defined soft-tissue mass and radiating spiculation of calcification within it.