title: The pharmacological treatment of aggressive fibromatosis: a systematic review.
abstract: BACKGROUND
  Despite the use of surgery and radiotherapy, 20-35% of patients with aggressive fibromatosis (AF) will have local recurrence. The purpose of this review was to collect and analyze all available information regarding the role of non-cytotoxic and cytotoxic chemotherapy in AF that has been accumulated over the past few decades.
  
  
  METHODS
  A systematic review of published clinical trials, studies and case series was carried out using the Medline Express Databases and the Cochrane Collaboration Database from 1970 to October 2000.
  
  
  RESULTS
  Most studies published in the literature are in the form of successful case reports and single-arm series with small patient numbers. Most commonly used agents include hormonal agents, non-steroidal anti-inflammatory drugs (NSAIDs), interferons and cytotoxics. The literature data support the use of hormonal agents. Several questions, however, remain unresolved, such as which is the most suitable endocrine manipulation and what is the optimal dose and duration of treatment. NSAIDs and interferons have demonstrated activity against AF either alone or in combination with hormone therapy or chemotherapy but the precise mechanism of action is still unknown. Finally, there is growing evidence in the literature that chemotherapy is effective against AF with almost one in two patients being likely to respond.
  
  
  CONCLUSIONS
  The evidence in the literature supports the opinion that both non-cytotoxic and cytotoxic chemotherapies are effective against AF. However, the lack of sufficient patient numbers and randomized trials compromises the validity of the reported results and mandates further investigation with properly designed prospective studies including larger patient numbers, with main end points to include not only tumor response rate and survival but also quality-of-life issues.
authors.affiliation: Social Security Organization Oncology Center, Kifissia, Greece. janinis@internet.gr
authors.forename: J
authors.initials: J
authors.lastname: Janinis
authors.forename: M
authors.initials: M
authors.lastname: Patriki
authors.forename: L
authors.initials: L
authors.lastname: Vini
authors.forename: G
authors.initials: G
authors.lastname: Aravantinos
authors.forename: J S
authors.initials: JS
authors.lastname: Whelan
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002986:Clinical Trials as Topic; D005350:Fibroma / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D011379:Prognosis; D013534:Survival
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D018931:Antineoplastic Agents, Hormonal