The first Scandinavian protocol for
Ewing's sarcoma, SSG IV, resulted in a local control rate of 74% and 5-year
metastasis-free survival (MFS) of 43%. The second protocol, SSG IX, was started in order to improve upon these results. It featured four
chemotherapy cycles, each consisting of two courses of VAI (
vincristine,
doxorubicin,
ifosfamide) alternating with one course of PAI (
cisplatin,
doxorubicin,
ifosfamide) at 3-weekly intervals. Total treatment time was 35 weeks. Local
therapy was given at week 9. Inoperable or non-radically operated patients received hyperfractionated accelerated
radiotherapy 1.5 Gy twice daily between
chemotherapy courses to a total dose of 42-60 Gy, depending on surgical radicality and tumour localisation. 88 patients were included (58 male, 30 female, mean age 20 years; range 5-65 years). The tumour (73 M0 and 15 M1) was located centrally in 31 patients (35%), in the extremities in 34 (39%) and other sites in 23 (26%) of cases. The median size of tumour was 10 cm (range 2-23), soft tissue was invaded in 87%. Surgery was the local
therapy for 60 (68%) patients:
amputation in 8 and local excision in 52. The
surgical margins were wide in 35 patients, marginal in 14 and intralesional in 3.
Radiotherapy was given to 17 non-radically operated patients postoperatively and to 28 patients with inoperable tumours primarily. Histological responses were evaluated in 52 patients. 9 local recurrences were observed (10%). Distant
metastases developed in 24 M0 patients (33%). The estimated 5-year MFS was 58% and overall survival (OS) 70% for M0 and 27% and 28% for M1 patients, respectively. Survival was favourable in patients with non-metastatic extremity tumours (90%) and tumours operated with wide margins (90%). Patients with a total
necrosis after
chemotherapy had a better OS than those with a partial or poor response (P=0.003). The toxicity (World Health Organisation) was acceptable (gastrointestinal G1-2; haematological G3-4). The
SSG IX protocol gave better local control and survival rates than the SSG IV. Whether this is due to a higher therapeutic efficacy of the present protocol cannot be ascertained in this comparison with a historical control.