title: [Chemotherapy in fibromatoses of childhood and adolescence: results from the Cooperative soft tissue sarcoma study (CWS) and the Italian Cooperative study group (ICG-AIEOP)].
abstract: 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.
  
  
  METHODS
  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.
authors.affiliation: Abteilung pädiatrische Onkologie und Hämatologie, Olgahospital Stuttgart. cws.study@olgahospital.s.shuttle.de
authors.forename: T
authors.initials: T
authors.lastname: Pilz
authors.forename: T B
authors.initials: TB
authors.lastname: Pilgrim
authors.forename: G
authors.initials: G
authors.lastname: Bisogno
authors.forename: R
authors.initials: R
authors.lastname: Knietig
authors.forename: E
authors.initials: E
authors.lastname: Koscielniak
authors.forename: M
authors.initials: M
authors.lastname: Carli
authors.forename: J
authors.initials: J
authors.lastname: Treuner
mesh_terms: D000293:Adolescent; D000328:Adult; D000903:Antibiotics, Antineoplastic / Q000008:administration & dosage; D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage; D000972:Antineoplastic Agents, Phytogenic / Q000008:administration & dosage; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D005260:Female; D005350:Fibroma / Q000188:drug therapy; D018222:Desmoid Tumors / Q000188:drug therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D015337:Multicenter Studies as Topic; D018224:Myofibromatosis / Q000188:drug therapy*; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D012509:Sarcoma / Q000188:drug therapy*; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000903:Antibiotics, Antineoplastic; D018906:Antineoplastic Agents, Alkylating; D000972:Antineoplastic Agents, Phytogenic