Bone sarcoma complicating Paget's disease.<i>A report of 3 cases with long survival</i>

Abstract

STEITIS DEFORMANS may be complicated by 0 superimposed bone sarcomas and this was appreciated by Paget in his original description of the disease in 1877.The number of patients with Paget's disease in any hospital population is difficult to ascertain because of the large number of asymptomatic cases.9The incidence of sarcoma arising in Paget's disease is therefore unknown but probably approaches the 0.9% calculated from a large number of cases at the Mayo Clinic.7 Patients with these tumors have been considered to have an ominous prognosis.Jaffe4 and Lichtenstein5 stated that they had not seen a cure.Porretta and co-workers7 in a review of the English literature were able to find only 1 reported cure and were unable to add any from the large experience of the Mayo Clinic, Rochester, Minn.In our recent similar review we were able to find 5 cases of apparent cure from this tumor (Table 2).A review of the cases of sarcoma arising in Paget's disease at the Massachusetts General Hospital, Boston, Mass., yielded 3 apparent cures in a series of 20 such patients.One case (1 9) has been previously reported.1In order to dispel the almost universal pessimism with which this tumor has been viewed, a detailed discussion of the 2 new cases, together with a brief summary of the previously reported case, seemed indicated.The present cases bring the total of reported cures oi sarcomas arising in Paget's disease to 7. CASE REPORTSFrom the files of the Massachusetts General Hospital from 1925 to 1960,20 cases of sarcoma arising in Paget's disease of bone were found (Table 1).In the first 5 cases, neither operative nor autopsy data are available, but the patients had unequivocal roentgenological