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Paget's Sarcoma
Introduction
Definition
Pagetic sarcoma is a rare anaplastic malignancy and one of the most serious complications of Paget's disease. It usually occurs in patients with polyostotic Paget's disease and it has a much poorer prognosis than primary osteosarcoma.
Prevelance and Epidemiology
Osteosarcoma is the most common tumor associated with Paget's disease. Approximately 1% of Paget's patients develop osteosarcoma-an increase in risk that is several thousand-fold higher than that of the general population (Hadjipavlou 1992). Some reports in the literature have placed the incidence at 5%, but lower frequencies are seen in reported studies in which both asymptomatic and symptomatic cases of Paget's disease are followed. (Hansen 1999)
Although the incidence of osteosarcoma in Paget's is relatively low (represents 3 to 18% of all osteogenic sarcomas), it is a major factor contributing to the morbidity and mortality of the disease, because of the high incidence of Paget's disease in the population (Polednak 1987). Twenty percent of the patients with osteosarcoma who are older than 40 years of age (Wick 1981) and as high as 50% of the patients with osteosarcoma over the age of 60 (Huvos 1986) have Paget's disease as a predisposing condition, making this a significant geriatric health risk (Hansen 1999).
In most series, the sex ratio is two men to one woman, similar to that of Paget's disease. In addition, according to the 238 reports in the literature, 79% had polyostotic and 21% had monostotic Paget's disease (Jattiot 1999).
Overview
The purpose of this chapter is to provide an overview of the anatomic considerations, natural history, clinical manifestations, diagnosis, prognosis and management of Paget's osteosarcoma.
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