Summary Although Paget's disease of bone is very common in the general population of New South Wales, sarcoma arising in bone affected by osteitis deformans is very rare and is considered to be less than 1 per cent. Twenty‐nine cases of sarcoma of this type were found in the records of the four teaching hospitals in Sydney during the last ten years. Included in this series are 5 personal cases which are reported in more detail. The commonest site of the primary malignant lesion was in the shoulder girdle (9 cases), a part of the skeleton not usually affected by Paget's disease in its early stages. Another striking feature was the development of multiple sarcomatous changes in bone and in six cases in this series these were noted in the skull. Although several patients developed sarcoma after a definite history of injury and some were associated with fractures when first noted, it was not considered that there was sufficient evidence to indicate the effect of trauma in determining a malignant change. It was noted that only one case of sarcoma developed after a subtrochanteric fracture, the commonest site for fractures in patients with Paget's disease, and no case developed after surgical intervention. In most cases where a biopsy or autopsy was made the tumour was described as an osteogenic sarcoma, but cases of fibrosarcoma and chondrosarcoma were also found in this series. Amputation of the lower limb was carried out in 7 patients and one forequarter amputation was performed. Only one of these patients lived more than a year after operation. Deep X‐ray was given in 16 cases, but appeared to have no effect and most patients died within a few months. There were two exceptions: a man of 56 years with a sclerosing osteogenic sarcoma of the upper end of the humerus lived three years and eight months, and a woman of 60 years with X‐ray evidence of a pathological fracture and a soft tissue tumour lived seven and a half years.

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The special features of fractures, usually in the femur or the tibia, and their treatment in Paget's disease of bone are discussed. Osteotomy of the femur or tibia for marked deformity, and joint replacement of the hip or knee for painful arthritis can be carried out quite successfully. The high incidence of sarcomas arising in the humerus, the development of multiple sites of malignant change, and some features of tumors on the skull are noted.