- 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
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
- But 75%in 12-25yrs of age
- Almost equal in both sexes, slightly more in males .
5. Epidemiology
- Hereditary form of retinoblastoma
- Li-Fraumeni syndrome (p53)
- Rothmund-Thomson syndrome (8q24)
6. Pathogenesis
- Modal incidence correlates with rapid bone growth
- Chemicals chlorantherene, AAF, Be compounds
7. Gross pathology
- Arise from multipotent mesenchymal cells
- Mixture of osteoid, fibrous, cartilaginous, necrotic,
- hemorrhagic, cystic areas
8. Gross pathology
- Extension into medullary cavity and subperiosteal
extension.
- Restricted bu periosteun and epiphyseal plate, but eventually
crosses it
9. Microscopic appearance
- Stroma - Malignant connective tissue with anaplastic spindle
cells
- Matrix of osteoid/fibrous/cartilagenous tissue
10. Classification
- PRIMARY OSTEOSARCOMAS are
- Conventional /classicosteosarcoma (highgrade, intra medullar
y)
- Low-grade intramedullary osteosarcoma
- High-grade surface osteosarcoma
- Telangiectatic osteosarcoma, and
11. Classification
- Osteosarcomas occurring at the site of another
diseaseprocess.
- more common in >50 years of age
- The most common causes are
- Previous radiation treatment
- Other associated conditions are
- Dedifferentiated chondrosarcomas
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
13. Skeletal distribution
- (sites of rapid bone growth)
- Metaphyseal(89%)>diaphyseal(10%)>epiphyseal(1%)
14. Clinical Presentation
- 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)
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
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
20. MRI
- best to detect extent into bone and soft tissues
21. CT
- 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
- 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)
25. Biopsy
- to conform the diagnosis.
26. Enneking staging system
- The staging system is typically depicted as follows
- Stage I: Low grade tumors
- Stage II: High grade tumors
- Stage III: Any tumors with evidence ofmetastasis
27. Differential Dx
28. Surface osteosarcoma
- High grade surface osteosarcoma
29. Parosteal
- Arises from surface,invade
- medullary cavity in late stages
- Large ossified mass in centre
30. Periosteal
- Arises from surface of diaphysis
- Most commonly femur and tibia
- Characterized by bony spicule formation
- Strands of osteoid producing spindle cells
- radiating between lobules of cartilage
31. High grade surface
- Appearance as parosteal but histology high grade and medullary
involvement more common.
32. Telangiectatic Osteosarcoma
- Presents with pathological fracture
- arises within the diaphysis
- Often entirely osteolytic
- Bone and cortex destruction
- 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
-
- Pts with pulmonary, non pulmonry (bone) or skip metastasis have
poor prognosis
-
- High grade tumor have poor prognosis
- Size of the primary lesion
-
- Large size tumors have worse prognosis then small size
tumors
-
- proximal tumors do worse than distal tumors.
- Secondary osteosarcoma:Poor prognosis
34. Treatment
- 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
- Post op chemo based on preop response
35. Chemotherapy
- Chemotherapy given preoperatively - Neoadjuvant
- Given postoperatively -Adjuvant
- Advantages ofneoadjuvantchemotherapy -
- 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)
- Actinomycin D (dactinomycin)
37. Surgery
- Themain goal of surgery is to safely and completely remove the
tumor.
- 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:
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
- 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
-
- 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
- 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.
- 5 year survival rate is 5% - 23%
48.