title: Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study.
abstract: BACKGROUND
  Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcoma with little evidence supporting treatment recommendations.
  
  
  METHODS
  Specialist centres collaborated to report prognostic factors and outcome for 113 patients.
  
  
  RESULTS
  Median age was 30 years (range: 11-80), male/female ratio 1.1. Primary sites were extremities (40%), trunk (47%) and head and neck (13%), 41 arising primarily in soft tissue. Seventeen patients had metastases at diagnosis. Mean follow-up was 14.9 years (range: 1-34), median overall survival (OS) 17 years (95% confidence interval (CI): 10.3-28.6). Ninety-five of 96 patients with localised disease underwent surgery, 54 additionally received combination chemotherapy. Sixty-five of 95 patients are alive and 45 progression-free (5 local recurrence, 34 distant metastases, 11 combined). Median progression-free survival (PFS) and OS were 7 (95% CI: 3.03-10.96) and 20 (95% CI: 12.63-27.36) years respectively. Chemotherapy administration in patients with localised disease was associated with reduced risk of recurrence (P=0.046; hazard ratio (HR)=0.482 95% CI: 0.213-0.996) and death (P=0.004; HR=0.445 95% CI: 0.256-0.774). Clear resection margins predicted less frequent local recurrence (2% versus 27%; P=0.002). Primary site and origin did not influence survival. The absence of metastases at diagnosis was associated with a significantly better outcome (P<0.0001). Data on radiotherapy indications, dose and fractionation were insufficiently complete, to allow comment of its impact on outcomes. Median OS for patients with metastases at presentation was 3 years (95% CI: 0-4.25).
  
  
  CONCLUSIONS
  Prognosis in MCS varies considerably. Metastatic disease at diagnosis has the strongest impact on survival. Complete resection and adjuvant chemotherapy should be considered as standard of care for localised disease.
authors.affiliation: The London Sarcoma Service, University College of London Hospital, London, United Kingdom; University Campus Bio-Medico, Rome, Italy.
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Frezza
authors.affiliation: Istituto Ortopedico Rizzoli di Bologna, Bologna, Italy.
authors.forename: Marilena
authors.initials: M
authors.lastname: Cesari
authors.affiliation: Bone Tumor Reference Centre at the Institute of Pathology, University Hospital Basel, Basel, Switzerland.
authors.forename: Daniel
authors.initials: D
authors.lastname: Baumhoer
authors.affiliation: Hospital Cochin, Paris, France.
authors.forename: David
authors.initials: D
authors.lastname: Biau
authors.affiliation: The Cooperative German-Austrian-Swiss Osteosarcoma Study Group (COSS Group), Germany.
authors.forename: Stephen
authors.initials: S
authors.lastname: Bielack
authors.affiliation: Azienda Ospedaliera Universitaria Careggi, Firenze, Italy.
authors.forename: Domenico Andrea
authors.initials: DA
authors.lastname: Campanacci
authors.affiliation: Coimbra University Medical Center, Coimbra, Portugal.
authors.forename: José
authors.initials: J
authors.lastname: Casanova
authors.affiliation: Leicester Royal Infirmary, Leicester, United Kingdom.
authors.forename: Claire
authors.initials: C
authors.lastname: Esler
authors.affiliation: Istituto Ortopedico Rizzoli di Bologna, Bologna, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Ferrari
authors.affiliation: Universitätsklinik für Orthopädie der Medizinischen, Universität Wien, Wien, Austria.
authors.forename: Philipp T
authors.initials: PT
authors.lastname: Funovics
authors.affiliation: Freeman Hospital, Newcastle, United Kingdom.
authors.forename: Craig
authors.initials: C
authors.lastname: Gerrand
authors.affiliation: The Royal Orthopaedic Hospital, Birmingham, United Kingdom.
authors.forename: Robert
authors.initials: R
authors.lastname: Grimer
authors.affiliation: Istituto Nazionale Tumori, Milano, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gronchi
authors.affiliation: Orthopaedic Hospital Gersthof, Wien, Austria.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Haffner
authors.affiliation: The Cooperative German-Austrian-Swiss Osteosarcoma Study Group (COSS Group), Germany.
authors.forename: Stefanie
authors.initials: S
authors.lastname: Hecker-Nolting
authors.affiliation: Bone Tumor Reference Centre at the Institute of Pathology, University Hospital Basel, Basel, Switzerland.
authors.forename: Sylvia
authors.initials: S
authors.lastname: Höller
authors.affiliation: The Royal Orthopaedic Hospital, Birmingham, United Kingdom.
authors.forename: Lee
authors.initials: L
authors.lastname: Jeys
authors.affiliation: University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Paul
authors.initials: P
authors.lastname: Jutte
authors.affiliation: Medical University of Graz, Graz, Austria.
authors.forename: Andreas
authors.initials: A
authors.lastname: Leithner
authors.affiliation: University of Navarra, Pamplona, Spain.
authors.forename: Mikel
authors.initials: M
authors.lastname: San-Julian
authors.affiliation: Norwegian Radium Hospital, Oslo, Norway.
authors.forename: Joachim
authors.initials: J
authors.lastname: Thorkildsen
authors.affiliation: University Campus Bio-Medico, Rome, Italy.
authors.forename: Bruno
authors.initials: B
authors.lastname: Vincenzi
authors.affiliation: Universitätsklinik für Orthopädie der Medizinischen, Universität Wien, Wien, Austria.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Windhager
authors.affiliation: The London Sarcoma Service, University College of London Hospital, London, United Kingdom. Electronic address: jeremy.whelan@uclh.nhs.uk.
authors.forename: Jeremy
authors.initials: J
authors.lastname: Whelan
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001859:Bone Neoplasms / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D002648:Child; D018211:Chondrosarcoma, Mesenchymal / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012955:Societies, Medical; D055815:Young Adult
keywords: Chemotherapy; Mesenchymal chondrosarcoma; Outcome