Osteosarcoma (1)
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Osteosarcoma (1)

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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 year...

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 year
Treated by chemo,amputation or rotationplasty

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Osteosarcoma (1) Osteosarcoma (1) Presentation Transcript

  • 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
  • Overview
    • Definition
    • Epidemiology
    • Pathogenesis
    • Skeletal distribution
    • Clinical presentation
    • Evaluation
    • Classification
    • Investigations
    • Treatment
    • Prognosis
  • What is osteosarcoma ?
    • Highly malignant tumor of mesenchymal origin.
    • Spindle shaped cells that produce osteoid.
    • 2 nd most common primary malignant bone tumor after MM.
  • 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 .
  • Epidemiology
    • Associated syndromes
    • Hereditary form of retinoblastoma
    • Li-Fraumeni syndrome (p53)
    • Rothmund-Thomson syndrome (8q24)
  • Pathogenesis
    • Unknown
    • Modal incidence correlates with rapid bone growth
    • Radiation >2000 rads
    • Chemicals – chlorantherene, AAF, Be compounds
  • Gross pathology
    • Arise from multipotent mesenchymal cells
    • Mixture of osteoid, fibrous, cartilaginous, necrotic,
    • hemorrhagic, cystic areas
    • Destruction of cortex
  • 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
  • Microscopic appearance
    • Stroma - Malignant connective tissue with anaplastic spindle cells
    • Matrix of osteoid/fibrous/cartilagenous tissue
  • Classification
    • PRIMARY or SECONDARY
    • PRIMARY OSTEOSARCOMAS are
    • Conventional /classic osteosarcoma (high grade, intra medullar y)
    • Low-grade intramedullary osteosarcoma
    • Parosteal osteosarcoma
    • Periosteal osteosarcoma
    • High-grade surface osteosarcoma
    • Telangiectatic osteosarcoma, and
    • Small cell osteosarcoma.
  • Classification
    • SECONDARY OSTEOSARCOMAS
    • Osteosarcomas occurring at the site of another disease process.
    • 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
  • 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
  • Skeletal distribution
    • Distal femur
    • Proximal tibia
    • Proximal humerus
    • (sites of rapid bone growth)
    • others
    • Metaphyseal(89%)>diaphyseal(10%)>epiphyseal(1%)
  • 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
  • Evaluation
    • Suspected diagnosis by history and physical examination
    • Supported by investigations
  • Plain X-ray (Most valuable)
    • sclerotic
    • Lytic
    Mixed (most common)
  • 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
  • Plain X-ray
    • Periosteal reaction may 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.
  • Other investigations
    • MRI
    • CT
    • Angiogram
    • Bone scan
    • Laboratory studies
    • Biopsy
  • MRI
    • best to detect extent into bone and soft tissues
  • CT
    • Not of much use
    • CT chest to detect lung metastasis
  • Angiogram
    • Determine vascularity of the tumour
    • Detect vascular displacement
    • Relationship of vessels to the tumour
  • 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
  • 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
  • Biopsy
    • to conform the diagnosis.
    • Types
    • Fine needle aspiration
    • Core needle biopsy
    • Open incisional biopsy
  • 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
  • Differential Dx
    • Giant Cell Tumor
    • Chondrosarcoma
    • Fibrosarcoma
    • Aneursymal Bone Cyst
    • Ewings sarcoma
    • Osteoblastoma
    • Metastasis
    • Lymphoma
    • Osteomyelitis
    • Chondroblastoma
    • Post traumatic callus
    • Other variants
  • Surface osteosarcoma
    • Parosteal
    • Periosteal
    • High grade surface osteosarcoma
  • 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
  • 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
  • High grade surface
    • Very rare
    • Age group 20-30’s
    • Appearance as parosteal but histology high grade and medullary involvement more common.
  • 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
  • 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
  • Treatment
    • Current standard of care
    • 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
  • 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 micrometastases 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
  • 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
  • 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.
  • Surgery choice
    • Surgical procedures fall into three basic categories:
    • Amputation
    • Limb salvage
    • Rotationplasty
  • 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.
    • Patient’s age, desired level of function, cosmetic preference and long-term prognosis must also be considered.
  • 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 patient’s return to a more comfortable and productive life.
  • Amputation
    • Indication
      • 1. Grossly displaced pathologic fracture
      • 2. Encasement of neurovascular bundle
      • 3. Tumor that enlarges during preop chemo and is adjacent to neurovascular bundle
      • 4. Palliative measure in metastatic disease
      • 5. If the tumor has caused massive necrosis, fungation, infection, or vascular compromise.
  • Limb salvage surgery
    • Removing the tumor with a normal cuff of tissue surrounding it while preserving vascular and nerve supply to the extremity.
    • 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
  • 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.
    • 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
  • 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)
  • 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%
    • THANK YOU !