Highly malignant tumor of mesenchymal origin.Spindle shaped cells that produce osteoid.2nd most common primary malignant bone tumor after MM.Incidence – 1 to 3 per million per yearTreated by chemo,amputation or rotationplasty
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1.Osteo = bone/osteoid tissue Sarcoma = malignant tumour of
connective tissue 02/04/12 Dr. Pruthviraj Nistane Deptt. Of
Orthopaedics Govt. Medical College and Rajindra Hospital,
Patiala
2. Overview
Definition
Epidemiology
Pathogenesis
Skeletal distribution
Clinical presentation
Evaluation
Classification
Investigations
Treatment
Prognosis
3. What is osteosarcoma ?
Highly malignant tumor of mesenchymal origin.
Spindle shaped cells that produce osteoid.
2 ndmost common primary malignant bone tumor after MM.
4. Epidemiology
Incidence 1 to 3 per million per year
Any age
But 75%in 12-25yrs of age
Almost equal in both sexes, slightly more in males .
5. Epidemiology
Associatedsyndromes
Hereditary form of retinoblastoma
Li-Fraumeni syndrome (p53)
Rothmund-Thomson syndrome (8q24)
6. Pathogenesis
Unknown
Modal incidence correlates with rapid bone growth
Radiation >2000 rads
Chemicals chlorantherene, AAF, Be compounds
7. Gross pathology
Arise from multipotent mesenchymal cells
Mixture of osteoid, fibrous, cartilaginous, necrotic,
hemorrhagic, cystic areas
Destruction of cortex
8. Gross pathology
Metaphyseal, Central.
Extension into medullary cavity and subperiosteal
extension.
Restricted bu periosteun and epiphyseal plate, but eventually
crosses it
Reactive periosteal
new bone formation
Metastasis lungs
9. Microscopic appearance
Stroma - Malignant connective tissue with anaplastic spindle
cells
Matrix of osteoid/fibrous/cartilagenous tissue
10. Classification
PRIMARY or SECONDARY
PRIMARY OSTEOSARCOMAS are
Conventional /classicosteosarcoma (highgrade, intra medullar
y)
Low-grade intramedullary osteosarcoma
Parosteal osteosarcoma
Periosteal osteosarcoma
High-grade surface osteosarcoma
Telangiectatic osteosarcoma, and
Small cell osteosarcoma.
11. Classification
SECONDARY OSTEOSARCOMAS
Osteosarcomas occurring at the site of another
diseaseprocess.
more common in >50 years of age
The most common causes are
Paget disease
Previous radiation treatment
Other associated conditions are
Fibrous dysplasia
Bone infarcts
Osteochondromas
Chronic osteomyelitis
Dedifferentiated chondrosarcomas
Osteogenesis imperfecta
12. Classic High Grade Osteosarcoma
These aggressive, high-grade tumors begin in an intramedullary
location, but may break through the cortex and form a soft-tissue
mass.
The histologic hallmark -malignant osteoblastic spindle cells
producing osteoid,presence of woven bone with malignant appearing
stromal cells
subtypes-
osteoblastic,
chondroblastic and
fibroblastic
13. Skeletal distribution
Distal femur
Proximal tibia
Proximal humerus
(sites of rapid bone growth)
others
Metaphyseal(89%)>diaphyseal(10%)>epiphyseal(1%)
14. Clinical Presentation
Pain progresssive pain
due to microinfarction
night pain in 25 %
Swelling-Palpable mass is noted in up to 1/3
of patients at the first visit
Fever, malaise or other constitutional symptoms
are not typical of osteosarcoma
15. Evaluation
Suspected diagnosis by history and physical examination
Supported by investigations
16. Plain X-ray(Most valuable)
sclerotic
Lytic
Mixed (most common) 17. Plain X-ray
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
Intra medullary
Borders are ill defined
18. Plain X-ray
Periosteal reactionmay 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.
19. Other investigations
MRI
CT
Angiogram
Bone scan
Laboratory studies
Biopsy
20. MRI
best to detect extent into bone and soft tissues
21. CT
Not of much use
CT chest to detect lung metastasis
22. Angiogram
Determine vascularity of the tumour
Detect vascular displacement
Relationship of vessels to the tumour
23. 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
24. laboratory studies
Full blood count, ESR, CRP.
LDH (elevated level is associated with poorprognosis)
ALP (highly osteogenic)
Platelet count
Electrolyte levels
Liver function tests
Renal function tests
Urinalysis
25. Biopsy
to conform the diagnosis.
Types
Fine needle aspiration
Core needle biopsy
Openincisional biopsy
26. 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 ofmetastasis
27. Differential Dx
Giant Cell Tumor
Chondrosarcoma
Fibrosarcoma
Aneursymal Bone Cyst
Ewings sarcoma
Osteoblastoma
Metastasis
Lymphoma
Osteomyelitis
Chondroblastoma
Post traumatic callus
Other variants
28. Surface osteosarcoma
Parosteal
Periosteal
High grade surface osteosarcoma
29. Parosteal
5% of osteosarcomas
Posterior metaphysis of
distal femur
Arises from surface,invade
medullary cavity in late stages
Tends to encircle bone
Low grade,Slow growing
Large ossified mass in centre
30. Periosteal
Arises from surface of diaphysis
Most commonly femur and tibia
Characterized by bony spicule formation
perpendicular to shaft
Strands of osteoid producing spindle cells
radiating between lobules of cartilage
Sunburst
Low grade
31. High grade surface
Very rare
Age group 20-30s
Appearance as parosteal but histology high grade and medullary
involvement more common.
32. Telangiectatic Osteosarcoma
Aggressive
Presents with pathological fracture
5% of all osteosarcomas
arises within the diaphysis
Radiology
Often entirely osteolytic
Bone and cortex destruction
Periosteal reaction
Codman's triangles
Pathology
Gross appearance is a multi-cystic similar to an aneurysmal
bone cyst.
Microscopically it has large blood filled spaces and thin
septation. Within the septa there is scanty osteoid production by
the pleomorphic malignant cells
33. 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
regression of the primary tumor, making a successful
limbsalvage 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 occurduring any
delaybefore surgery.
Given for about 3-4 weeks before definitive procedure
36. 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
37. Surgery
Themain 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.
38. Surgery choice
Surgical procedures fall into three basic categories:
Amputation
Limb salvage
Rotationplasty
39. 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.
Patients age, desired level of function, cosmetic preference
and long-term prognosis must also be considered.
40. 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 patients return to a more
comfortableand productive life.
41. Amputation
Indication
1. Grossly displaced pathologic fracture
2. Encasement of neurovascular bundle
3. Tumor that enlarges during preop chemo andis adjacent to
neurovascular bundle
4. Palliative measure in metastatic disease
5. If the tumor has caused massive necrosis,fungation,
infection, or vascular compromise.
42. Limb salvage surgery
Removing the tumor with a normal cuff of tissue surrounding it
while preserving vascular and nerve supply to the extremity.
43.
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
44. 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.
45.
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
46. 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)
47. 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.