[Sarcomas associated with Paget's disease].

The authors report 12 cases of Paget's disease with sarcomatous degeneration that were observed among 311 patients with Paget's disease hospitalized in the Montpellier Rheumatology Clinic. They compare their experience with information they were able to extract from the literature in French and English. The frequency of such degeneration in cases of Paget's disease is difficult to determine but did not appear to exceed 1%. Degeneration rarely occurred before the age of 50 years, affected men twice as frequently as women, and occurred particularly in cases of diffuse Paget's disease, mainly in the femur or the humerus; the rachis was rarely affected. Pain was the main symptom, was practically constant, was remarkable because of its permanence and its intensity. Tumefaction was frequently seen. Pathological fractures were seen in almost a third of the patients with sarcomas of the long bones. Radiculo-medullary compression characterized the rare cases with involvement of the vertebral column or the sacrum. Radiography showed rupture of cortical layers with invasion of the soft parts without any periostal reaction. Histological investigation confirmed the diagnosis by demonstrating one of the three types of osteogenic sarcoma: osteosarcomas were the most frequent. Less frequently giant-cell sarcomas or reticulosarcomas were found. The affected patients nearly always died, survival at five years being rare. Treatment, amputation or radiotherapy, was disappointing.