Chemotherapy in fibromatoses of childhood and adolescence: Experiences of the German Cooperative Soft Tissue Sarcoma Study (CWS) and the Italian Cooperative Study Group (ICG-AIEOP)

Background: Fibromatoses are a group of semimalign 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 20(th) week of treatment. The histological subgroup can provide good information on the relapse rate and aggressivity to be expected