Osteosarcoma (knee joint)

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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

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.NPRIMARY 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 3
rd
&

4
th
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.

30. PAROSTEAL OSTEOSARCOMA

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 OsteomyelitisEwing’s
sarcoma
Site Metaphyseal Metaphysis Diaphysis
Locationjuxtacortical
centric
Eccentric
juxtacorticalcentric

39. Periosteal reaction & Zone of
transition
osteosarcoma OsteomyelitisEwing’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.

41. Thank You