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

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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.

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