Results of Treatment for Soft Tissue Sarcoma in Childhood and Adolescence: A Final Report of the German Cooperative Soft Tissue Sarcoma Study CWS-86

Abstract

The improved design of the study incorporating risk-adapted radiotherapy allowed treatment to be reduced for selected groups of patients without compromising survival.

Higher rates of metastatic disease may explain the declining trend in Swedish paediatric rhabdomyosarcoma survival rates

“…The prognosis regarding sex, age, pathological subtype and size was in accordance with earlier studies 9, 16, 18. Just as the report by Dantonello et al 16 showed, the primary site category with the best prognosis was the orbit and the worst was the category called other sites.…”

“…As expected, there was a slight male predominance and the age distribution was comparable to international data published, as was the distribution of pathological subtype, size and primary site 3, 6, 7, 8, 9, 10. The frequency of metastatic disease for the total cohort was equivalent to that reported in earlier publications 5, 7, 8, 9, 10, but during the last time period, 2000–2010, the metastasis rate at diagnosis was surprisingly high. During the last time period, the rate of metastatic disease was significantly higher than the earlier time periods, with a metastasis rate of 28% compared to 9% in 1984–1989 and 13% in 1990–1999.…”

“…However, there was a significant increase in survival rates between the first two periods. Compared with earlier publications, the outcomes from 1984 to 1989 were not very good, with a 5‐year overall survival of 59 ± 7% 2, 7, 9. A report from the Nordic Society of Paediatric Haematology and Oncology, which provided data from 1991–1995 and 1996–2000, showed 5‐year overall survival rates of 68% and 75% respectively 2.…”

“…During the two last periods, 1990–1999 and 2000–2010, all but one of the patients received systemic treatment with multi‐agent conventional chemotherapy according to the respective CWS protocol. The prescribed chemotherapeutical regimens were vincristine, actinomycin D, ifosfamide and doxorubicin (VAIA) for all patients in CWS‐86 9 and vincristine, actinomycin D, cyclophosphamide and doxorubicin (VACA) or etoposide, vincristine, actinomycin D, ifosfamide and doxorubicin (EVAIA) for localised RMS in CWS‐91 16. Patients with metastatic disease in CWS‐91 were recommended treatment according to the protocol of the International Society of Paediatric Oncology.…”

“…There is a slight predominance in boys 3, 5, 6, 7, 8, who also have a better prognosis 1, 9. It is a rare disease in adults, and the median age is 5 years of age 7, 10.…”