Osteosarcoma in Paget Disease

• Occurs in < 1% of all Paget's patients
• Paget’s: 30 X increase in incidence of bone sarcoma
• Sarcomatous change usually occurs in diffuse, long standing, polyostotic Paget's disease
• Sarcomatous change most common in pelvis, humerus, femur
• 50% are osteosarcoma
  • Usually osteolytic
  • Also - fibrosarcoma, chondrosarcoma, giant cell tumor

• Treatment
  • Palliative resection
  • Chemotherapy
• Prognosis
  • Grave, with survival > 2 years rare

 
Osteosarcoma in Paget Disease. Frontal radiograph of pelvis demonstrates Paget's Disease of right hemipelvis as evidenced by thickened cortex and prominent trabeculae with large, lytic, geographic lesion in iliac crest representing an osteolytic osteosarcoma