title: Chemotherapy in patients with desmoid tumors: a study from the French Sarcoma Group (FSG).
abstract: BACKGROUND
  Data regarding the role of chemotherapy (CT) in patients with recurrent and/or unresectable desmoid tumors (DTs) are scarce.
  
  
  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.
authors: Department of Medical Oncology, Institut Bergonie, Bordeaux.
authors: D
authors: D
authors: Garbay
authors: Department of Medicine, Institut Gustave Roussy, Villejuif.
authors: A
authors: A
authors: Le Cesne
authors: Department of Medical Oncology, Centre Oscar Lambret, Lille.
authors: N
authors: N
authors: Penel
authors: Department of Medical Oncology, Institut Claudius Regaud, Toulouse.
authors: C
authors: C
authors: Chevreau
authors: Institut d'hématologie et d'oncologie pédiatrique, Hospices civils de Lyon et Centre Léon Bérard, Lyon.
authors: P
authors: P
authors: Marec-Berard
authors: Department of Medical Oncology, Centre Léon Bérard, Lyon.
authors: J-Y
authors: JY
authors: Blay
authors: Department of Medical Oncology, Institut Bergonie, Bordeaux.
authors: M
authors: M
authors: Debled
authors: Department of Medical Oncology, Centre Georges-François Leclerc, Dijon.
authors: N
authors: N
authors: Isambert
authors: Department of Medical Oncology, Centre Antoine-Lacassagne, Nice.
authors: A
authors: A
authors: Thyss
authors: Department of Medical Oncology, Centre René Gauducheau, Nantes.
authors: E
authors: E
authors: Bompas
authors: Department of Medical Oncology, Institut de Cancérologie de la Loire, Saint-Priest-en-Jarez.
authors: O
authors: O
authors: Collard
authors: Department of Medical Oncology, Hôpital La Timone, Marseille.
authors: S
authors: S
authors: Salas
authors: Department of Pathology, Institut Bergonie, Bordeaux, France.
authors: J-M
authors: JM
authors: Coindre
authors: Department of Medical Oncology, Institut Bergonie, Bordeaux.
authors: B
authors: B
authors: Bui
authors: Department of Medical Oncology, Institut Bergonie, Bordeaux. Electronic address: italiano@bergonie.org.
authors: A
authors: A
authors: Italiano
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018943:Anthracyclines / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D018222:Desmoid Tumors / Q000188:drug therapy* / Q000401:mortality; D005602:France; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D014747:Vinblastine / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D018943:Anthracyclines; D014747:Vinblastine; D008727:Methotrexate