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The 5-year survival for metastatic Paget's sarcoma is less than 10%
8%
153/1831
Less than 1% of patients with Paget's disease develop secondary Paget's sarcoma
7%
125/1831
While osteosarcoma is the most common histologic sub-type of Paget's sarcoma, fibrosarcoma and chondrosarcoma sub-types also occur
5%
92/1831
Paget's sarcoma typically occurs in patients over 50 years of age
2%
39/1831
Treatment of Paget's sarcoma is via surgery alone
77%
1414/1831
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Paget's sarcoma is a rare secondary sarcoma which occurs in patients with Paget's disease (Illustration A). Less than 1% of patients with Paget's disease ever develop Paget's sarcoma. It typically presents with progressive pain, soft tissue swelling, or pathological fracture. Typically patients with Paget's sarcoma are older at diagnosis (average 55-80 years). Paget's sarcoma is a very aggressive secondary sarcoma which is histologically similar to osteosarcoma, however fibrosarcoma and chondrosarcoma variants do exist. Treatment consists of surgery with chemotherapy vs. palliative radiotherapy in patients too infirm to withstand surgery or chemotherapy. The 5-year survival for metastatic Paget's sarcoma is less than 10%. Mankin and Hornicek review the Mass General experience and report on 43 patients with Paget's sarcoma. Their results are a compilation of two cohorts, the first treated from 1942 to 1967, and the second from 1972 to 2001. As such, they conclude that despite multiple different treatment protocols, the overall survival of Paget's sarcoma is poor.
3.9
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