Osteogenic sarcoma associated with Paget's disease of bone. A clinicopathologic study of 65 patients

Abstract

Among 1177 osteogenic sarcoma patients diagnosed and treated at Memorial Hospital, 65 (5.5%) were associated with either monostotic or polyostotic Paget's disease. The overall median age was 64 years (range, 39-82 years). In those patients older than 40 years of age, the frequency of sarcomatous transformation rose to 27%. There were slightly more men (55%) than women. The most common skeletal sites were the pelvic bones (34%), the humerus (22%), the femur (19%), and the craniofacial bones (14%). Unrelenting pain and tender swelling were the most common presenting symptoms (85%), with pathologic fracture in 14 (22%) patients. In two-thirds of the cases, the radiographic presentation was that of a lytic destructive lesion; while in the others it showed a sclerotic, mixed, or permeative character. In almost one-half of the cases, the histologic appearance of the osteogenic sarcomas was either fibrohistocytomatous or osteoblastic. In spite of radical surgical amputations, only three patients survived longer than 5 years. The prognosis of Paget's sarcoma is significantly less favorable than in osteogenic sarcoma arising de novo in patients of comparable age.