Osteosarcoma

Background

• Most common primary pediatric bone tumor
  • 3% of all childhood cancers
• Around two-thirds of those diagnosed with non-metastatic disease under the age of 40 will be long-term survivors or cured of the disease with appropriate treatment
• Survival is less than 20 percent with metastatic disease
  • One-fifth will have metastases at presentation
  • Most common metastatic site is lung followed by bone
• Bimodal as it peaks in early adolescence and over the age of 65

Pathophysiology

• Overproduction of osteoid and immature bone by malignant osteoblasts

Clinical Features

• Often an adolescent during a growth spurt
• Bone pain for several months that worsens with activity and is more painful at night
• Absence of constitutional symptoms (e.g., fevers, weight loss, night sweats, and decreased appetite are typically absent)
• Large soft tissue mass on exam that is tender to palpation
• Occurs at metaphysis of long bones
  • Most commonly at the distal femur followed by the proximal tibia
• Less frequently presents as pathological fracture
• Risk factors include prior cancer treatment, Paget disease of bone, benign bone disease, Hereditary Retinoblastoma or Li-Fraumeni syndrome