Results of treatment for soft tissue sarcoma in childhood and adolescence: a final report of the German Cooperative Soft Tissue Sarcoma Study CWS-86.

PURPOSE: The goal of the second German Soft Tissue Sarcoma Study CWS-86 (1985 to 1990) was to improve the prognosis in children and adolescents with soft tissue sarcoma by means of a clinical trial comprising intensive chemotherapy and risk-adapted local therapy. PATIENTS AND METHODS: There were 372 eligible patients. A staging system based on the postsurgical extent of disease was used. Chemotherapy consisted of vincristine, dactinomycin, doxorubicin, and ifosfamide. Radiotherapy was administered early at 10 to 13 weeks simultaneously with the second chemotherapy cycle (32 Gy or 54. 4 Gy). The single dose was reduced to 1.6 Gy and given twice daily (accelerated hyperfractionation). RESULTS: The event-free survival (EFS) and overall survival rates at 5 years were 59% +/- 3% and 69% +/- 3%, respectively. The 5-year EFS rate according to stage was as follows: stage I, 83% +/- 5%; stage II, 69% +/- 6%; stage III, 57% +/- 4%; and stage IV, 19% +/- 6%. The outcome for patients with stage III disease who required radiotherapy was much better in the CWS-86 study compared with the CWS-81 study (5-year EFS, 60% +/- 5% v 44% +/- 6%; P =.053). The most common treatment failure was isolated local relapse, with 14% of patients relapsing at the primary tumor site. CONCLUSION: The improved design of the study incorporating risk-adapted radiotherapy allowed treatment to be reduced for selected groups of patients without compromising survival.

[Chemotherapy in fibromatoses of childhood and adolescence: results from the Cooperative soft tissue sarcoma study (CWS) and the Italian Cooperative study group (ICG-AIEOP)]. / Chemotherapie bei Fibromatosen im Kindes- und Jugendalter: Erfahrungen der Cooperativen Weichteilsarkom Studie (CWS) und Italienischen Cooperativen Studiengruppe (ICG--AIEOP).

BACKGROUND: Fibromatoses are a group of semimalignant tumors which grow infiltratively without metastases. If radical surgery is not at all possible or only by mutilation the question of neoadjuvant concepts arises. PATIENTS: We report on therapy and outcome of 36 patients registered to the German CWS study group and the Italian ICG study group for soft tissue sarcoma who were between 0 and 23 years of age. 15 had a histology of infantile (myo-) fibromatosis (9 unifocal, 6 multifocal). 15 patients had desmoid-like, aggressive fibromatosis. METHODS: We performed a retrospective analysis of the patients registered to the CWS and ICG study group. For literature review a medline search was performed. RESULTS: 23 patients received chemotherapy with anthracyclines (VAIA) or without (VAC) or monotherapy with alkylating agents. In 19 cases response to chemotherapy was measurable. In 9 patients tumors responded to chemotherapy. In some cases response was evident only after 20 weeks of treatment. In the group of non responders treatment was stopped early (after 4 weeks) in some cases. Relapses occurred mainly in the group of desmoid-like, aggressive fibromatosis (7 out of 15). In the group of infantile (myo-) fibromatosis only one patient suffered a relapse (1 out of 9). The literature review showed that mainly ADR/DTIC, VAC and MTX/VBL was used for inoperable fibromatoses. Concerning the response rates there where no major differences. Concerning toxicity the regimen with methotrexate and vinblastine seems superior to the others. CONCLUSIONS: As a chemotherapy regimen of first choice vinblastine with low dose methotrexate can be recommended. The therapy should not be stopped before the 20th week of treatment. The histological subgroup can provide good information on the relapse rate and aggressivity to be expected.