title: Primary cardiac sarcomas: a retrospective study of the French Sarcoma Group.
abstract: BACKGROUND
  Primary cardiac sarcomas (PCS) are rare tumours of dismal prognosis.
  
  
  METHODS
  Data of 124 patients with PCS referred to institutions of the French Sarcoma Group (FSG) from 1977 and 2010 were reviewed.
  
  
  RESULTS
  Median age was 48.8years. PCS were poorly-differentiated sarcomas (N=45, 36.3%), angiosarcomas (N=40, 32.3%), leiomyosarcomas (N=16, 12.9%) and others (N=23, 18.6%). At diagnosis, 100 patients (80.6%) were localised and 24 (19.4%) metastatic. Tumours were located in the right (N=47, 38.8%), left atrial cavities (N=45, 37.2%) or encompassed several locations in nine cases (7.4%). Surgery was performed in 81 cases (65.3%). Heart transplant was performed in five patients. Radiotherapy adjuvant (N=18, 14.5%) or alone (N=6, 4.8%) was performed in non-metastatic patients only (N=24, 19.4%). With a median follow-up of 51.2months, median overall survival (OS) was 17.2months for the entire cohort, 38.8months after complete resection versus 18.2 after incomplete resection and 11.2months in non-resected patients. Radiotherapy was associated with improved progression-free survival (PFS) on multivariate analysis. Chemotherapy was significantly associated with better OS only in non-operated patients but not in operated patients. In non-metastatic patients, surgery (hazard ratio [HR]=0.42, p<0.001), male gender (HR=0.56, p=.032) was associated with better OS and surgery (HR=0.61; p=.076), radiotherapy (HR=0.43; p=.004) and chemotherapy (HR=0.30, p=.003) improved PFS.
  
  
  CONCLUSIONS
  Only surgical resection is associated with a perspective of prolonged survival. Chemotherapy is associated with a better outcome in non-resected patients.
authors.affiliation: Centre Georges-François Leclerc, Dijon, France. Electronic address: nisambert@cgfl.fr.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Isambert
authors.affiliation: Centre Léon Bérard, Lyon, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Ray-Coquard
authors.affiliation: Institut Bergonie, Bordeaux, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Italiano
authors.affiliation: Centre Alexis Vautrin, Nancy, France.
authors.forename: Maria
authors.initials: M
authors.lastname: Rios
authors.affiliation: Centre Eugène Marquis, Rennes, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Kerbrat
authors.affiliation: Centre Georges-François Leclerc, Dijon, France.
authors.forename: Mélanie
authors.initials: M
authors.lastname: Gauthier
authors.affiliation: Institut Claudius Regaud, Toulouse, France.
authors.forename: Aurélien
authors.initials: A
authors.lastname: Blouet
authors.affiliation: CHRU Jean Minjoz, Besançon, France.
authors.forename: Loïc
authors.initials: L
authors.lastname: Chaigneau
authors.affiliation: Hôpital La Timone, Marseille, France.
authors.forename: Florence
authors.initials: F
authors.lastname: Duffaud
authors.affiliation: Institut Curie, Paris, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Piperno-Neumann
authors.affiliation: Hôpital de Hautepierre, Strasbourg, France.
authors.forename: Jean-Emmanuel
authors.initials: JE
authors.lastname: Kurtz
authors.affiliation: Hôpital Louis Pradel, Lyon, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Girard
authors.affiliation: Institut de Cancérologie Lucien Neuwirth, Saint-Priest-en-Jarez, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Collard
authors.affiliation: Institut de Cancérologie de l'Ouest René Gauducheau, Saint Herblain, France.
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Bompas
authors.affiliation: Centre Oscar Lambret, Lille, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Penel
authors.affiliation: Hôtel-Dieu, Clermont-Ferrand, France.
authors.forename: Jacques-Olivier
authors.initials: JO
authors.lastname: Bay
authors.affiliation: Centre Henry Becquerel, Rouen, France.
authors.forename: Cécile
authors.initials: C
authors.lastname: Guillemet
authors.affiliation: Centre Georges-François Leclerc, Dijon, France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Collin
authors.affiliation: Centre Léon Bérard, Lyon, France.
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Blay
authors.affiliation: Institut Gustave Roussy, Villejuif, France.
authors.forename: Axel
authors.initials: A
authors.lastname: Le Cesne
authors.affiliation: Centre Antoine Lacassagne, Nice, France.
authors.forename: Juliette
authors.initials: J
authors.lastname: Thariat
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005602:France; D006338:Heart Neoplasms / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012509:Sarcoma / Q000473:pathology* / Q000628:therapy*; D015996:Survival Rate; D055815:Young Adult
keywords: Cardiac; Chemotherapy; Radiotherapy; Sarcoma; Surgery