Neoplasms arising in Paget's disease of bone: a study of 82 cases

Eighty-two cases of neoplasms arising in Paget's disease of bone from the Mid-America Bone Tumor Registry, accessioned between 1958 and 1983, were reviewed. There were 77 osteosarcomas, 3 fibrosarcomas, 1 chondrosarcoma, and 1 giant cell tumor. The male/female ratio was 2:1. The age distribution was 18%, 29%, 36%, and 17% for the groups less than 51 years, 51-60 years, 61-70 years, and greater than 70 years, respectively. The femur was involved by tumor in 22%; the humerus in 21%; the pelvis in 21%; the calvarium in 12%; the tibia in 10%; and other bones in 15% of the cases. There were 48% survivors after 1 year; 17% after two; and 5% after three and five years; two additional patients with tumor lived for more than seven and 11 years. Radiographs available for simultaneous study in 43 cases revealed predominant tumor patterns that were mixed in 69%, osteoblastic in 21%, and osteolytic in 10%. Other radiographic data and the clinical and histopathologic data are in agreement with five earlier major studies. While the prognosis of neoplasms arising in Paget's disease generally is not good, a small fraction of long-term survivors after aggressive therapy makes this complication a challenge for timely radiographic diagnosis and histopathologic confirmation.