Chemotherapy in patients with desmoid tumors: a study from the French Sarcoma Group (FSG)

Ann Oncol. 2012 Jan;23(1):182-186. doi: 10.1093/annonc/mdr051. Epub 2011 Mar 28.


Background: Data regarding the role of chemotherapy (CT) in patients with recurrent and/or unresectable desmoid tumors (DTs) are scarce.

Patients and methods: Records of patients with DT who were treated with CT in centers from the French Sarcoma Group were reviewed.

Results: Sixty-two patients entered the study. The two most common locations were extremities (35.5%) and internal trunk (32.5%). Twelve patients (19.5%) were diagnosed with Gardner syndrome. Thirty-seven patients (54.7%) received previously one or more lines of systemic therapies (nonsteroidal anti-inflammatory drugs: 43.5%, antiestrogens: 43.5% and imatinib: 30.5%). Combination CT was delivered in 44 cases (71%) and single agent in 18 patients (29%), respectively. Thirteen patients (21%) received an anthracycline-containing regimen. The most frequent nonanthracycline regimen was the methotrexate-vinblastine combination (n=27). Complete response, partial response, stable disease and progressive disease were observed in 1 (1.6%), 12 (19.4%), 37 (59.6%) and 12 (19.4%) patients, respectively. The response rate was higher with anthracycline-containing regimens: 54% versus 12%, P=0.0011. Median progression-free survival (PFS) was 40.8 months. The sole factor associated with improved PFS was the nonlimb location: 12.1 months (95% confidence interval 5.6-18.7) versus not reached, P=0.03.

Conclusions: CT has significant activity in DT. Anthracycline-containing regimens appear to be associated with a higher response rate.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Anthracyclines / therapeutic use
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Child
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Fibromatosis, Aggressive / drug therapy*
  • Fibromatosis, Aggressive / mortality
  • France
  • Humans
  • Kaplan-Meier Estimate
  • Male
  • Methotrexate / therapeutic use
  • Middle Aged
  • Vinblastine / therapeutic use
  • Young Adult


  • Anthracyclines
  • Vinblastine
  • Methotrexate