title: Therapy of aggressive fibromatosis is still an open question: a series of patients treated at a single institution.
abstract: OBJECTIVE
  This study was an attempt to evaluate the possible role of chemo-hormonotherapy as a possible approach in managing inoperable, deep extra-abdominal aggressive fibromatosis.
  
  
  METHODS
  A series of patients with inoperable, deep extra-abdominal aggressive fibromatosis, were treated with combination chemo-hormotherapy. Therapy consisted of 6 cycles standard CVP (cyclophosphamide 750 mg/m(2) and vincristine 1.2 mg/m(2), given bolus intravenously (i.v.) on day 1, plus prednisone 40 mg/m(2)/day, days 1-5, every 3 weeks) and tamoxifen 20 mg daily.
  
  
  RESULTS
  From 1995 to 2004, 9 patients, concomitantly, without selection, were included in this investigation. Their median age was 24 years (range 18-47), with predominantly male sex (6/9). Extremities were the most frequent localization (5/9), followed by chest wall in 3 and abdominal wall in one patient. Tumor size in most patients was 5-10 cm, and 3 patients had bulky disease (over 10 cm). Five patients had undergone previous surgery (3 wide excisions and 2 palliative interventions). Complete remission (CR) was observed in one patient, partial remission (PR) in 4 and stabilization of disease (SD) in 4 patients. In responders, the median duration to the onset of response was 10 months (range 4-14); median response duration was 32+ months (range 14-82). No relapse of disease was observed up until now.
  
  
  CONCLUSIONS
  Systemic treatment should be considered in patients with aggressive fibromatosis for whom local treatment approaches are not possible or have failed. All patients should be included in clinical trials.
authors.affiliation: Department of Clinical Oncology, Institute for Oncology & Radiology of Serbia, Belgrade, Serbia and Montenegro.
authors.forename: D
authors.initials: D
authors.lastname: Ristić
authors.forename: Z
authors.initials: Z
authors.lastname: Tomasević