title: Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor).
abstract: BACKGROUND
  In the current study, the authors examined the outcomes of patients with desmoid tumors who received systemic therapy at a single institution to provide a basis for the examination of newer agents.
  
  
  METHODS
  Records of patients with desmoid tumors who were treated with chemotherapy at the study institution were reviewed. The activity of nonsteroidal anti-inflammatory drugs was not addressed. Patients without measurable disease and those receiving therapy could not be documented, and those receiving prophylactic therapy were excluded.
  
  
  RESULTS
  A total of 68 patients received 157 lines of therapy. At the time of last follow-up, 9 patients had died, 7 of progressive disease. The cohort was 62% female, with a median age of 32.5 years. Approximately 32% of the patients had Gardner syndrome. The median follow-up was 63 months, and patients received a median of 2 lines of therapy. An intra-abdominal primary tumor location was the most common (44%). The greatest Response Evaluation Criteria in Solid Tumors (RECIST) response rate was observed with anthracyclines and hormonal therapy and the lowest response was noted with single-agent dacarbazine/temozolomide or tyrosine kinase inhibitors, principally imatinib. On multivariate analysis, macroscopic nodular morphology and the presence of Gardner syndrome were the only tumor factors found to be associated with a greater time to disease progression.
  
  
  CONCLUSIONS
  Compared with other agents, antiestrogens and anthracycline-containing regimens appear to be associated with a higher radiological response rate against desmoid tumors. Systemic therapy can be successful in patients with desmoid tumors, and is a viable option in lieu of morbid or disabling surgery.
authors.affiliation: Department of Medicine, Memorial Sloan-Kettering Cancer Center, New York, New York 10065-6007, USA.
authors.forename: Veridiana Pires
authors.initials: VP
authors.lastname: de Camargo
authors.forename: Mary L
authors.initials: ML
authors.lastname: Keohan
authors.forename: David R
authors.initials: DR
authors.lastname: D'Adamo
authors.forename: Cristina R
authors.initials: CR
authors.lastname: Antonescu
authors.forename: Murray F
authors.initials: MF
authors.lastname: Brennan
authors.forename: Samuel
authors.initials: S
authors.lastname: Singer
authors.forename: Linda S
authors.initials: LS
authors.lastname: Ahn
authors.forename: Robert G
authors.initials: RG
authors.lastname: Maki
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D018450:Disease Progression; D005260:Female; D018222:Desmoid Tumors / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000401:mortality; D006801:Humans; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012008:Recurrence; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents