Osteosarcoma

Osteosarcoma
Updated: 09/24/2018
© Jun Wang, MD, PhD

General features
  • Any malignant tumors that produce osteoid directly from tumor cells
  • Most common primary bone tumor after myeloma
  • Young age, 10-25
  • Most common metastasis site: Lung
  • Rare metastasis to lymph nodes
Risk factors
If > 40
  • Paget disease
  • Radiation exposure
  • Thorotrast
  • Childhood chemotherapy
  • Other bone disorders such as fibrous dysplasia, osteochondromatosis, chondromatosis, etc
Clinical presentations
  • Most common clinical presentation: Localized pain
  • Mass
Most common site
  • Metaphysis of long bones, especially around the knee
  • Medullary cavity
Key pathogenesis
  • Unclear, probably associated with rapid bone growth
Key laboratory findings
  • Elevated alkaline phosphatase
Key radiological findings
  • Large, destructive mass
  • Lytic or blastic
  • Permeative margins
  • Sunburst pattern due to bone formation
  • Codman’s triangle: Shadow between cortex and raised ends of periosteum (due to reactive bone formation), non-specific (can be seen in Ewing sarcoma)
Key morphological features
  • Most commonly metaphysis
  • Bulky invasive mass with hemorrhage and necrosis
  • Pleomorphic tumor cells with osteoid matrix
Genetic abnormalities
  • Combined p53 and Rb mutation common
  • P53 mutations if multicentric


Negative prognosis indicator
  • Paget disease
  • Telangiectatic histology
  • Elevated serum alkaline phosphatase
  • Minimal postchemotherapy tumor necrosis
  • Involvement of craniofacial bones (not jaw) or vertebrae
  • Multifocal tumor
  • Loss of heterozygosity of RB gene
Treatment
  • Surgery
  • Chemotherapy


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