title: Gemcitabine in advanced angiosarcoma: a retrospective case series analysis from the Italian Rare Cancer Network.
abstract: BACKGROUND
  Angiosarcoma is a highly aggressive soft tissue sarcoma. Responses to anthracyclines plus/minus ifosfamide, and taxanes alone or in combination with gemcitabine are well documented. Very few data are available on gemcitabine as a single agent.
  
  
  METHODS
  We retrospectively reviewed all cases of advanced progressive angiosarcoma treated with gemcitabine as a single agent (1000 mg/m(2) i.v. every week for 3 weeks every 4 weeks), at Istituto Nazionale Tumori and within the Italian Rare Cancers Network from January 2008 to November 2010.
  
  
  RESULTS
  Twenty-five patients [mean age: 52 years; radiation therapy (RT)-related: 8] received gemcitabine. Best tumor response by RECIST was as follows: complete response = 2, partial response = 14, stable disease = 2, progressive disease = 7 cases, for an overall response rate (PR + CR) of 68%. Six of eight post-RT angiosarcomas responded to treatment. Median overall survival (OS) was 17 months. Median progression-free survival (PFS) was 7 months (range 1-40 months). One patient with a locally advanced thyroid angiosarcoma became resectable after 5 months of gemcitabine, with <10% residual viable tumor cells seen on surgical specimen. Overall, gemcitabine was well tolerated.
  
  
  CONCLUSIONS
  Gemcitabine is active in both RT- and non-RT-related angiosarcoma, with dimensional and possibly long-lasting responses. A formal phase II study on gemcitabine as a single agent is warranted.
authors.affiliation: Department of Cancer Medicine, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Istituto Nazionale dei Tumori, Milano, Italy. silvia.stacchiotti@istitutotumori.mi.it
authors.forename: S
authors.initials: S
authors.lastname: Stacchiotti
authors.forename: E
authors.initials: E
authors.lastname: Palassini
authors.forename: R
authors.initials: R
authors.lastname: Sanfilippo
authors.forename: B
authors.initials: B
authors.lastname: Vincenzi
authors.forename: M G
authors.initials: MG
authors.lastname: Arena
authors.forename: A M
authors.initials: AM
authors.lastname: Bochicchio
authors.forename: P
authors.initials: P
authors.lastname: De Rosa
authors.forename: A
authors.initials: A
authors.lastname: Nuzzo
authors.forename: S
authors.initials: S
authors.lastname: Turano
authors.forename: C
authors.initials: C
authors.lastname: Morosi
authors.forename: A P
authors.initials: AP
authors.lastname: Dei Tos
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; D000369:Aged, 80 and over; D003841:Deoxycytidine / Q000031:analogs & derivatives* / Q000627:therapeutic use; D006394:Hemangiosarcoma / Q000188:drug therapy*; D006801:Humans; D008875:Middle Aged; D012189:Retrospective Studies; D016019:Survival Analysis; D000093542:Gemcitabine
chemical_list: D003841:Deoxycytidine; D000093542:Gemcitabine