PAGET'S SARCOMA OF BONE

Abstract

1. An analytical study of eighty histologically proven cases of Paget's sarcoma confirms and elaborates existing knowledge of the etiology of this tumour. The frequency with which this tumour occurs in certain parts of England may be seen by comparison with the numbers of osteosarcoma and fibrosarcoma cases registered at Bristol and Leeds. An overall incidence figure for Paget's sarcoma of 0·l6 and 0·18 per 100,000 population per annum has been calculated for the Bristol and Leeds areas respectively. The similarity of the age incidence curves of Paget's disease and Paget's sarcoma are shown and discussed in the light of demographic information. 2. Histologically, most Paget's sarcomata resemble recognised tumour types as seen in otherwise normal bones, the most frequent forms being osteosarcoma and fibrosarcoma. The type of tumour arising in an osteitic bone is uninfluenced by age, sex or site of origin. Thirty-eight per cent of the cases were complicated by fracture, which occurred with slightly greater frequency in fibrosarcoma and with advancing age. A causal connection between sarcoma and fracture is supported but not vice versa. 3. Survival statisticsfor this series are compared with Bristol records for osteosarcoma and fibrosarcoma (without Paget's disease), the mean survival of seventy-four patients being 11·1 months from the date of the initial symptoms of malignancy. Four long-term survivors are reported, the five-year and ten-year survival rates being respectively 5 per cent and 3·1 per cent. The effects of age, site, sex, fracture and treatment upon survival are tabulated and discussed.