title: Response to chemotherapy of solitary fibrous tumour: a retrospective study.
abstract: BACKGROUND
  To report on anthracycline-based chemotherapy in a retrospective case-series analysis of solitary fibrous tumour (SFT) patients treated within the Italian Rare Cancer Network.
  
  
  METHODS
  We reviewed a set of SFT treated with chemotherapy since 2002, focusing on anthracycline, administered alone or in combination with ifosfamide. Responses to ifosfamide as a single agent were also evaluated. Pathologic diagnosis was centrally reviewed, distinguishing typical, malignant (MSFT) and dedifferentiated (DSFT) subtypes.
  
  
  RESULTS
  Among 42 SFT patients treated with chemotherapy, we selected 31 cases (mean age: 62 years; locally advanced/metastatic: 13/18; front-line/further line: 25/6; typical/MSFT/DSFT/not assessable: 1/17/12/1) who received anthracycline-based chemotherapy (anthracycline monotherapy: eight; anthracycline+ifosfamide: 23). 30 patients are evaluable for response. Best response by Response Evaluation Criteria in Solid Tumours (RECIST) was: partial response (PR): 6 (20%), stable disease (SD): eight (27%), progressive disease (PD): 16 (53%) cases. Responses were confirmed after 3 months. Median progression-free survival (PFS) was 4 (range 2-15) months, with 20% of patients being progression-free at 6 months. PR was found in 2/18 (11%) MSFT and 4/12 (30%) DSFT, with a median PFS of 3.5 and 5 months in MSFT and DSFT, respectively. 19 patients received high-dose prolonged-infusion ifosfamide (front-line/further line: 11/8; typical/MSFT/DSFT: 0/15/4) with two (10%) PR, five (26%) SD, 12 (63%) PD.
  
  
  CONCLUSIONS
  This retrospective series suggests that in SFT anthracyclines have a degree of antitumour activity in the range of soft tissue sarcoma chemotherapy. Ifosfamide monotherapy seemed to have lower activity. A higher response rate was observed in DSFT in comparison to MSFT. Studies on targeted therapies are ongoing.
authors.affiliation: Adult Sarcoma Medical Oncology Unit, Department of Cancer Medicine, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy. silvia.stacchiotti@istitutotumori.mi.it
authors.forename: S
authors.initials: S
authors.lastname: Stacchiotti
authors.forename: M
authors.initials: M
authors.lastname: Libertini
authors.forename: T
authors.initials: T
authors.lastname: Negri
authors.forename: E
authors.initials: E
authors.lastname: Palassini
authors.forename: A
authors.initials: A
authors.lastname: Gronchi
authors.forename: S
authors.initials: S
authors.lastname: Fatigoni
authors.forename: P
authors.initials: P
authors.lastname: Poletti
authors.forename: B
authors.initials: B
authors.lastname: Vincenzi
authors.forename: A P
authors.initials: AP
authors.lastname: Dei Tos
authors.forename: L
authors.initials: L
authors.lastname: Mariani
authors.forename: S
authors.initials: S
authors.lastname: Pilotti
authors.forename: P G
authors.initials: PG
authors.lastname: Casali
mesh_terms: D000328:Adult; D000368:Aged; D018943:Anthracyclines / Q000008:administration & dosage / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D054364:Solitary Fibrous Tumors / Q000188:drug therapy* / Q000473:pathology; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D018943:Anthracyclines; D007069:Ifosfamide