Osteo = bone/osteoid tissue
Sarcoma = malignant tumor of connective tissue
04/28/14 1
Overview
Definition
Epidemiology
Pathogenesis
Skeletal distribution
Clinical presentation
Evaluation
Classification...
What is osteosarcoma ?
 Highly malignant tumor arising from primitive
mesenchymal bone-forming cells.
Histologic hallmar...
• Any bone can be involved but the Mc sites are – distal
femur, proximal tibia, proximal humerus.
• Mc metastasis to the l...
Distal femur
Proximal tibia
Proximal humerus
(sites of rapid bone growth)
 others
Metaphyseal(89%)>diaphyseal(10%)>e...
Etiology
Rapid bone growth – adolescence growth spurt in the
metaphyseal area near the growth plate.
Radiation exposure ...
Classification
PRIMARY or SECONDARY
PRIMARY OSTEOSARCOMAS (15 – 25 yrs)
 Conventional /classic osteosarcoma (high
grade...
Classification
SECONDARY OSTEOSARCOMAS
 Osteosarcoma occurring at the site of another disease
process.
 more common in ...
1. Conventional type: osteoblastic, chondroblastic &
fibroblastic OS
2. Telangiectatic or osteolytic type OS
3. Small c...
Gross pathology
10
Osteoblastic tumor – grayish white hard & gritty
feeling when cut.
Chondroid type – opalescent & blui...
Histologic appearance
Stroma - Malignant
connective tissue with
anaplastic spindle cells in
mesenchymal parenchyma.
Tumo...
Clinical Presentation
Pain– progressive pain particularly with activity.
Swelling - Palpable mass in the region of metap...
Investigations:
Radiological examination
- plain X-rays
- MRI
- CT scan
- bone scan
• Laboratory studies – serum alkaline...
Plain X-ray
scleroticLytic
14
Mixed (most
common)
Plain X-ray
Irregular destruction in metaphysis.
New bone formation.
Periosteal reaction.
Sunray appearance or hair on...
16
Codman triangle is a term
used to describe the
triangular area of
subperiosteal bone that is
created when a lesion, of...
MRI :
• used to know the soft tissue extant.
CT:
• Not of much use in osteosarcoma.
 CT chest to detect lung metastasis
17
Angiogram
• Determine vascularity of the tumour
• Detect vascular displacement
• Relationship of vessels to the tumour
18
Bone scan
A bone scan to look for skeletal
metastases or multi focal disease
Thallium scan - Monitor effects of
chemothe...
laboratory studies
Full blood count, ESR, CRP.
LDH (elevated level is associated with
poor prognosis)
ALP (highly osteo...
Biopsy
to conform the diagnosis.
Types
 Fine needle aspiration
 Core needle biopsy
 Open incisional biopsy
21
Enneking staging system
The staging system is typically depicted as follows
Stage I: Low grade tumors
I-A intra compartme...
Differential Dx
Giant Cell Tumor
Chondrosarcoma
Fibrosarcoma
Aneursymal Bone Cyst
Ewings sarcoma
Osteoblastoma
Meta...
Telangiectatic OS
Painful radiologically lytic mass
lesion in the metaphyseal portion
of long bones.
Mc site – distal fe...
Parosteal OS
Arise from the periosteum.
Mc occurrence in adults.
Mc site Posterior metaphysis of
distal femur.
slow gr...
Periosteal OS
Arises from diaphysis of long bones.
Mc femur & tibia
Presents with swelling & tenderness.
25% pathologi...
Prognostic Factors
Extent of the disease
Pts with pulmonary, non pulmonry (bone) or skip metastasis have
poor prognosis
...
Treatment
Radiological staging
Biopsy to confirm diagnosis
Preoperative chemotherapy
Repeat radiological staging (access c...
Chemotherapy
Chemotherapy given preoperatively -Neoadjuvant
Given postoperatively - Adjuvant
Advantages of neoadjuvant ...
Chemotherapy
The drugs used most often to treat
osteosarcoma are:
Methotrexate with leucovorin (folinic acid)
Doxorubicin...
Surgery
The main goal of surgery is to safely and
completely remove the tumor.
Historically – amputation.
Over the past...
Limb salvage surgery
Removing the tumor with a wide margin of
normal tissue surrounding it while preserving
vascular and ...
The skeletal defect after limb salvage procedure must be
reconstructed by :
Autologous bone graft.
Allograft
 Endopros...
Rotationplasty
compromise between amputation and limb salvage
mc used for osteosarcoma of the distal femur in
skeletally...
35
Radiotherapy
Radiation therapy has no major role in
osteosarcoma
Radiation therapy may be useful in some cases
where the...
Immunotherapy
Portion of the tumour is implanted in sarcoma
survivor & is removed after 14 days.
The sensitised lymphocy...
Follow up and Prognosis
Signs of recurrence, metastasis and treatment
related complications
Physical examination,radiogr...
THANK YOU !
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  1. 1. Osteo = bone/osteoid tissue Sarcoma = malignant tumor of connective tissue 04/28/14 1
  2. 2. Overview Definition Epidemiology Pathogenesis Skeletal distribution Clinical presentation Evaluation Classification Investigations Treatment Prognosis 2
  3. 3. What is osteosarcoma ?  Highly malignant tumor arising from primitive mesenchymal bone-forming cells. Histologic hallmark is production of malignant osteoid. Mc histological form of primary bone cancer . 2nd most common primary malignant bone tumor after MM. 3
  4. 4. • Any bone can be involved but the Mc sites are – distal femur, proximal tibia, proximal humerus. • Mc metastasis to the lungs through blood stream. INCIDENCE: More prevalent in males than female. Involves any age but highest occurrence in adolescence i.e. 12-25yrs. 4
  5. 5. Distal femur Proximal tibia Proximal humerus (sites of rapid bone growth)  others Metaphyseal(89%)>diaphyseal(10%)>epiphyseal(1%) 5 Skeletal distribution
  6. 6. Etiology Rapid bone growth – adolescence growth spurt in the metaphyseal area near the growth plate. Radiation exposure – mostly causes secondary forms. Genetic predisposition :  - hereditary form of retinoblastoma( RB gene mutation)  - Li-Fraumeni syndrome (p53 gene mutation)  - Rothmund – Thomson syndrome ( autosomal recessive) Paget's disease of bone – mostly secondary forms. 6
  7. 7. Classification PRIMARY or SECONDARY PRIMARY OSTEOSARCOMAS (15 – 25 yrs)  Conventional /classic osteosarcoma (high grade, intra medullar y)  Low-grade intramedullary osteosarcoma  Paraosteal osteosarcoma  Periosteal osteosarcoma  High-grade surface osteosarcoma  Telangiectatic osteosarcoma, and  Small cell osteosarcoma. 7
  8. 8. Classification SECONDARY OSTEOSARCOMAS  Osteosarcoma occurring at the site of another disease process.  more common in >50 years of age most commonly a/w premalignant condition like - Paget disease - Previous radiation treatment - endochondromatosis - Fibrous dysplasia - Osteochondromas - Osteogenesis imperfecta 8
  9. 9. 1. Conventional type: osteoblastic, chondroblastic & fibroblastic OS 2. Telangiectatic or osteolytic type OS 3. Small cell OS 4. Low grade central OS 5. Periosteal OS 6. Paraosteal OS 7. Secondary OS 8. High grade surface OS 9. Extra skeletal OS 9 HISTOPATHOLOGIC VARIENTS:
  10. 10. Gross pathology 10 Osteoblastic tumor – grayish white hard & gritty feeling when cut. Chondroid type – opalescent & bluish grey. Fibroblastic – typical fish flesh sarcomatous appearance. Telangiectatic – areas of tumor necrosis & blood filled spaces.
  11. 11. Histologic appearance Stroma - Malignant connective tissue with anaplastic spindle cells in mesenchymal parenchyma. Tumor cells surrounded by osteoid matrix c/f Osteoblastic – lot of new bone  fibrous – fibroblasts Chondroid - cartilaginous tissue 11
  12. 12. Clinical Presentation Pain– progressive pain particularly with activity. Swelling - Palpable mass in the region of metaphysis. - skin over the swelling shiny with prominent veins. - swelling may be warm & tender. Decreased range of motion of the involved joint. Lymphadenopathy – unusual focal & regional lymph node involvement. Respiratory finding – late stage with lung metastasis. Fever & night sweats are rare. 12
  13. 13. Investigations: Radiological examination - plain X-rays - MRI - CT scan - bone scan • Laboratory studies – serum alkaline phosphatase. • Biopsy – core biopsy, FNAC • Angiogram 13
  14. 14. Plain X-ray scleroticLytic 14 Mixed (most common)
  15. 15. Plain X-ray Irregular destruction in metaphysis. New bone formation. Periosteal reaction. Sunray appearance or hair on end - tumor grows into the overlying soft tissue. Codman’s triangle – area off sub periosteal new bone formation seen at the area of tumor -host cortex junction. 15
  16. 16. 16 Codman triangle is a term used to describe the triangular area of subperiosteal bone that is created when a lesion, often a tumor, raises the periosteum away from the bone.
  17. 17. MRI : • used to know the soft tissue extant. CT: • Not of much use in osteosarcoma.  CT chest to detect lung metastasis 17
  18. 18. Angiogram • Determine vascularity of the tumour • Detect vascular displacement • Relationship of vessels to the tumour 18
  19. 19. Bone scan A bone scan to look for skeletal metastases or multi focal disease Thallium scan - Monitor effects of chemotherapy & Detect local recurrence of tumor 19
  20. 20. 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 20
  21. 21. Biopsy to conform the diagnosis. Types  Fine needle aspiration  Core needle biopsy  Open incisional biopsy 21
  22. 22. 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 22
  23. 23. Differential Dx Giant Cell Tumor Chondrosarcoma Fibrosarcoma Aneursymal Bone Cyst Ewings sarcoma Osteoblastoma Metastasis Lymphoma Osteomyelitis Chondroblastoma Post traumatic callus Other variants 23
  24. 24. Telangiectatic OS Painful radiologically lytic mass lesion in the metaphyseal portion of long bones. Mc site – distal femur. Histology - Blood filled vascular spaces lined by osteoblasts. Presents with local pain, soft tissue mass & fractures. d/d aneurysmal bone cyst. Mets through hematogenous route to bones & lung. 24
  25. 25. Parosteal OS Arise from the periosteum. Mc occurrence in adults. Mc site Posterior metaphysis of distal femur. slow growing & late to metastasize. Better prognosis. Large ossified mass in centre. 25
  26. 26. Periosteal OS Arises from diaphysis of long bones. Mc femur & tibia Presents with swelling & tenderness. 25% pathological fractures. X-ray shows sunburst or hair on end periosteal reaction. Histology – lobular & chondroblastic matrix. Intermediate prognosis. 26
  27. 27. 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 27
  28. 28. Treatment 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 28
  29. 29. 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 micro metastases 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 29
  30. 30. Chemotherapy The drugs used most often to treat osteosarcoma are: Methotrexate with leucovorin (folinic acid) Doxorubicin (Adriamycin) Cisplatin or carboplatin Citrovorum factor. Etoposide Cyclophosphamide Actinomycin D Bleomycin 30
  31. 31. 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. 31
  32. 32. Limb salvage surgery Removing the tumor with a wide margin of normal tissue surrounding it while preserving vascular and nerve supply to the extremity. 32
  33. 33. The skeletal defect after limb salvage procedure must be reconstructed by : Autologous bone graft. Allograft  Endoprosthesis (most common)– replacing the removed bone with a metal implant. Rotationoplasty- after tumor rresection the distal portion of the leg is rotated 180dgrees & reattached to the thigh at the proximal edge of resection. 33
  34. 34. Rotationplasty compromise between amputation and limb salvage mc used for osteosarcoma 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. For e.g. 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. 34
  35. 35. 35
  36. 36. 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) 36
  37. 37. Immunotherapy Portion of the tumour is implanted in sarcoma survivor & is removed after 14 days. The sensitised lymphocytes from the survivor are infused into the patient. These cells selectively kill the cancer cells. 37
  38. 38. 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% 38
  39. 39. THANK YOU ! 39

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