Bone sarcomas in Paget disease

Abstract

The most serious complication of Paget disease is the development of sarcoma. Forty-one cases of bone sarcoma in Paget disease, which represented our institution's entire experience, were reviewed. The relative frequency of sarcomatous degeneration was 0.9%. Histologically, 35 of 41 lesions were osteogenic sarcomas, and six were fibrosarcomas. The distribution of lesions was similar to that expected with uncomplicated Paget disease, except for a disproportionately high number of lesions in the humerus; only one lesion involved the vertebrae. The tumors were categorized radiographically as lytic, mixed, and sclerotic, in descending order of frequency. The vast majority of lesions occurred in the pelvis, humerus, and femur. Overall prognosis for these patients was poor, with a five-year survival of 8%. Finding areas of cortical destruction is particularly useful in the detection of malignant degeneration. The radiologist must maintain a high index of suspicion when evaluating radiographs of patients with Paget disease, especially of those who present with pain or a palpable mass.