osteosarcoma (1)

Download Osteosarcoma (1)

Post on 07-May-2015

13.241 views

Category:

Health & Medicine

5 download

Embed Size (px)

DESCRIPTION

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

TRANSCRIPT

  • 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