Secondary osteosarocma is a rare but serious complication of paget’s disease and paget’s osteosarcoma mainly affect elderly population. Most recent study by Mirabello et al11 shows that age-adjusted incidence rates in 0-24 years age group is 4.4 per million, which represents approximately 53% of all reported osteosarcoma cases with only 1 case of Paget’s osteosarcoma in this age group.
Individuals ages 25 to 59 years had the lowest incidence rates of osteosarcoma (1.7 per million) that included 4 cases of osteosarcoma in Paget’s disease. Osteosarcoma in elderly (>60 years old) represented approximately 19% of all reported osteosarcoma cases (4.2 per million) with 62 cases of osteosarcoma occurring in Pagets disease11.
Although the overall survival rate for secondary osteosarcomas is arguable similar to primary osteosarcomas 12-14, paget’s osteosarcoma remains an incurable disease with a poor prognosis. Shaylor et al15 reported in their study of 26 patient with osteosarcoma in Paget’ s disease .
The survival rate was 53% at 1 year, 25% at 2 years and no patient survived for 5 years despite modern methods of management of bone tumors. All patients died of metastatic disease. The median survival was 21 months for those treated with curative intent and 7 months for those treated palliatively. Four of the five patients treated with limb sparing surgery developed local recurrence between 5 and 12 months, the fifth died at 14 months. There was no difference in survival between amputation and limb salvage.
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