Nicolas Isambert1, Isabelle Ray-Coquard2, Antoine Italiano3, Maria Rios4, Pierre Kerbrat5, Mélanie Gauthier6, Aurélien Blouet7, Loïc Chaigneau8, Florence Duffaud9, Sophie Piperno-Neumann10, Jean-Emmanuel Kurtz11, Nicolas Girard12, Olivier Collard13, Emmanuelle Bompas14, Nicolas Penel15, Jacques-Olivier Bay16, Cécile Guillemet17, Françoise Collin6, Jean-Yves Blay2, Axel Le Cesne18, Juliette Thariat19. 1. Centre Georges-François Leclerc, Dijon, France. Electronic address: nisambert@cgfl.fr. 2. Centre Léon Bérard, Lyon, France. 3. Institut Bergonie, Bordeaux, France. 4. Centre Alexis Vautrin, Nancy, France. 5. Centre Eugène Marquis, Rennes, France. 6. Centre Georges-François Leclerc, Dijon, France. 7. Institut Claudius Regaud, Toulouse, France. 8. CHRU Jean Minjoz, Besançon, France. 9. Hôpital La Timone, Marseille, France. 10. Institut Curie, Paris, France. 11. Hôpital de Hautepierre, Strasbourg, France. 12. Hôpital Louis Pradel, Lyon, France. 13. Institut de Cancérologie Lucien Neuwirth, Saint-Priest-en-Jarez, France. 14. Institut de Cancérologie de l'Ouest René Gauducheau, Saint Herblain, France. 15. Centre Oscar Lambret, Lille, France. 16. Hôtel-Dieu, Clermont-Ferrand, France. 17. Centre Henry Becquerel, Rouen, France. 18. Institut Gustave Roussy, Villejuif, France. 19. Centre Antoine Lacassagne, Nice, France.
Abstract
INTRODUCTION: 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. CONCLUSION: Only surgical resection is associated with a perspective of prolonged survival. Chemotherapy is associated with a better outcome in non-resected patients.
INTRODUCTION: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. CONCLUSION: Only surgical resection is associated with a perspective of prolonged survival. Chemotherapy is associated with a better outcome in non-resected patients.
Authors: Juan C Espinoza-Cobos; Sergio Ortiz-Obregón; Enrique Blanco-Lemus; Gerardo Gutiérrez-Tovar; Luis E Santos-Martínez; Luis A Moreno-Ruiz Journal: Arch Cardiol Mex Date: 2021