LONG-TERM OUTCOME OF PATIENTS WITH MONOSTOTIC EWINGS-SARCOMA TREATED WITH COMBINED-MODALITY

One hundred and twenty-one consecutive patients with monostotic Ewing' s sarcoma (ES) were treated according to three consecutive combined mo dality programs from 1974 to 1986. Their 3-year progression free survi val (PFS) rate from diagnosis of 59% was identical to the event free s urvival (EFS) rate, since all the 50 events occurring within 3 years f rom diagnosis were tumor recurrences. Primary tumor was treated with r adiotherapy in 75 cases, surgical resection plus radiotherapy in 38, a nd radical surgery in 8. Chemotherapy was given to all patients and ea ch program included adriamycin, vincristine, and cyclophosphamide +/- dactinomycin. Median follow-up was 12 years, ranging from 6 to 19 year s. The PFS rate decreased to 49% at 6 years and plateaued at 46% after the 7th year from diagnosis, even though some relapses were observed as late as 14 years from diagnosis. Second malignancies developed in 7 patients free from progressive ES and were represented by osteogenic sarcoma in previously irradiated bone in 4 cases and by breast carcino ma in 3. No other event but tumor relapse or second malignancy occurre d in this series. EFS rate was 47% at 6 years and 39% at 12 years, fur ther decreasing in the following years because of a number of late eve nts. A continuous PFS longer than 7 years may be consistent with cure in the majority of patients with monostotic ES. However, these patient s should be followed indefinitely because of risk of second malignanci es. (C) 1994 Wiley-Liss, Inc.