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Tumour
Osteosarcoma

Osteosarcoma

AKA Osteogenic Sarcoma

Definition

Primary malignant bone-producing bone tumour characterised by production of osteoid
- ie bone matrix produced by malignant cells

Epidemiology

Second most common malignant neoplasm of bone after multiple myeloma
20% of all malignant neoplasms of bone
Bimodal peak
- Teens
- Elderly
M:F – 1.5:1

Classification

Anatomical Classification

1. Central
- Further subclassified by
  - Grade
    - High grade (10-20yo, most common)
    - Low grade
  - Pathology
    - Conventional
    - Telangiectatic
    - Small cell
    - Giant cell
2. Juxtacortical (surface osteosarcoma)
- Low grade (30-40yo, usually low grade)
- High grade

Parosteal or Periosteal

3. Intra-cortical
4. Soft tissue
5. Secondary

Aetiology

Strong genetic influence
- Increased risk in children with familial retinoblastoma (500x)
- RB 1 gene on chromosome 13
- P53 on 17p
Also associated with chromosomal abnormalities such as
- Rothmund-Thompson syndrome
- Bloom syndrome