title: Anthracycline-based chemotherapy in extraskeletal myxoid chondrosarcoma: a retrospective study.
abstract: BACKGROUND
  Extraskeletal myxoid chondrosarcoma (EMC) is a rare subgroup within soft tissue sarcomas. Its sensitivity to chemotherapy is reported to be low.
  
  
  METHODS
  We retrospectively reviewed a series of 11 EMC patients treated as from 2001 within the Italian Rare Cancer Network (RCN) with anthracycline-based chemotherapy. Pathologic diagnosis was centrally reviewed in all cases and confirmed by the presence of the specific chromosomal rearrangements, involving the NR4A3 gene locus on chromosome 9.
  
  
  RESULTS
  Eleven patients treated with anthracycline-based chemotherapy were included (M/F: 9/2 - mean age: 52 years - site of primary: lower limb/other = 9/2 - metastatic = 11 - front line/ further line = 10/1 - anthracycline as single agent/ combined with ifosfamide = 1/10). Ten patients are evaluable for response. Overall, best response according to RECIST was: partial response (PR) = 4 (40 %), stable disease (SD) = 3, progressive disease (PD) = 3 cases. Median PFS was 8 (range 2-10) months.
  
  
  CONCLUSIONS
  By contrast to what reported so far, anthracycline-based chemotherapy is active in a distinct proportion of EMC patients.
authors.affiliation: Adult Mesenchymal Tumor Medical Oncology Unit, Cancer Medicine Department, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy. silvia.stacchiotti@istitutotumori.mi.it.
authors.forename: Silvia
authors.initials: S
authors.lastname: Stacchiotti
authors.forename: Gian Paolo
authors.initials: GP
authors.lastname: Dagrada
authors.forename: Roberta
authors.initials: R
authors.lastname: Sanfilippo
authors.forename: Tiziana
authors.initials: T
authors.lastname: Negri
authors.forename: Isabella
authors.initials: I
authors.lastname: Vittimberga
authors.forename: Stefano
authors.initials: S
authors.lastname: Ferrari
authors.forename: Federica
authors.initials: F
authors.lastname: Grosso
authors.forename: Gaetano
authors.initials: G
authors.lastname: Apice
authors.forename: Marco
authors.initials: M
authors.lastname: Tricomi
authors.forename: Chiara
authors.initials: C
authors.lastname: Colombo
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gronchi
authors.forename: Angelo P
authors.initials: AP
authors.lastname: Dei Tos
authors.forename: Silvana
authors.initials: S
authors.lastname: Pilotti
authors.forename: Paolo G
authors.initials: PG
authors.lastname: Casali