Sarcomatous degeneration in Paget's disease of bone

Sarcomatous degeneration is a rare but serious complication of Paget's disease of bone with an incidence of 0.1–1%. The true aetiology of Paget's sarcoma remains unclear. The most common sites for Paget's sarcoma are femur, humerus, pelvis, skull and tibia. Sarcomatous degeneration rarely occurs before the age of 50 years affecting men twice as often as women usually in patients with diffuse, polyostotic Paget's disease. Progressively increasing pain or new pain is the main presenting symptom. Pathological fractures occur in almost a third of long bone cases. The radiographic presentation is most commonly lytic. Histologically, approximately 50% of the lesions represent osteosarcomas. Patients with Paget's sarcoma have a worse prognosis than primary osteosarcoma in spite of recent advancements in therapeutic strategies including surgery, radiation therapy and chemotherapy.