Total prothesis in treatment of pagetic arthropathy

Abstract

Osteosarcoma arising in pagetic bone is rare, estimated to occur in less than 1% of persons with Paget’s disease of bone (PDB) and accounting for a small percentage of those osteosarcomas occurring in adults >40 years of age. Consistent with the distribution of PDB in the population, pagetic osteosarcomas tend to arise in elderly persons >60 years of age, and in white more than black males. These cancers show a predilection for the same bones affected by PDB except to note an increased incidence in the humerus and infrequent appearance in the spine. Neoplastic transformation appears to arise equally in both monostotic and polyostotic PDB, and is associated with variable elevations in the serum alkaline phosphatase. Osteogenic osteosarcomas predominate, and are seen by histopathology as arising in pagetic bone marked by the presence of large spindle cells, osteoid and chaotic bone remodeling. Osteoclast-like giant cells similar to those seen in pagetic bone may be seen infiltrating the tumor. These cancers may present as synchronous or metachronous lesions. Although there is clearly an increased risk for osteosarcoma in Paget’s disease, the molecular basis for this remains unclear. Relative expression levels of a number of genes representing key signaling pathways have been examined in Paget’s disease and pagetic osteosarcoma. New insights from the analysis of cellular constituents of pagetic osteosarcomas as well as from pagetic bone are changing the way we think about the pathogenesis of this disease.