Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study
Abstract Five‐year overall survival (OS) of localized RMS exceeds 70% in children (<18) but is very poor in adult patients. We analyzed the outcome and prognostic factors (PF) of a national series of adult patients with RMS in a large study. The study population consisted of two different cohorts...
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Wiley
2018-08-01
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| Online Access: | https://doi.org/10.1002/cam4.1374 |
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| author | Emmanuelle Bompas Loïc Campion Antoine Italiano Axel Le Cesne Christine Chevreau Nicolas Isambert Maud Toulmonde Olivier Mir Isabelle Ray‐Coquard Sophie Piperno‐Neumann Esma Saada‐Bouzid Maria Rios Jean‐Emmanuel Kurtz Corinne Delcambre Pascale Dubray‐Longeras Florence Duffaud Marie Karanian François Le Loarer Patrick Soulié Nicolas Penel Jean‐Yves Blay |
| author_facet | Emmanuelle Bompas Loïc Campion Antoine Italiano Axel Le Cesne Christine Chevreau Nicolas Isambert Maud Toulmonde Olivier Mir Isabelle Ray‐Coquard Sophie Piperno‐Neumann Esma Saada‐Bouzid Maria Rios Jean‐Emmanuel Kurtz Corinne Delcambre Pascale Dubray‐Longeras Florence Duffaud Marie Karanian François Le Loarer Patrick Soulié Nicolas Penel Jean‐Yves Blay |
| author_sort | Emmanuelle Bompas |
| collection | DOAJ |
| description | Abstract Five‐year overall survival (OS) of localized RMS exceeds 70% in children (<18) but is very poor in adult patients. We analyzed the outcome and prognostic factors (PF) of a national series of adult patients with RMS in a large study. The study population consisted of two different cohorts: a retrospective cohort (157 adult patients treated in 13 reference centers between 05/1981 and 02/2010) and the prospective cohort (292 patients with RMS diagnosed and treated between 01/2010 and 12/2014 in France) included in the NetSarc database. A descriptive analysis of patients’ characteristics and prognostic factors was conducted on both series which were compared. In the retrospective series, histological subtypes were embryonal (E‐RMS) for 21% of patients, alveolar (A‐RMS) for 35% of patients, and “adult‐type” P‐RMS (pleomorphic, spindle cell RMS, not otherwise specified) (P) for 44% patients. This distribution significantly differed in the prospective cohort: A‐RMS: 18%; E‐RMS: 17%; and P‐RMS 65%. With a median follow‐up of 8.5 years, 5‐year OS for localized RMS and advanced RMS (with nodes and/or metastases) was 43% and 5%, respectively, (P < 0.0001), and median OS was 51, 33, and 16 months for E‐RMS, A‐RMS, and P‐RMS, respectively, in the retrospective cohort. The median OS was less than 40 months for the prospective nationwide cohort for the entire population. In a multivariate analysis of the retrospective study, independent prognostic factors for OS were A‐RMS, R0 resection, and adjuvant radiotherapy (RT). For localized RMS, age and use of pediatric chemotherapy (CT) regimen are independent prognostic factors. Adult patients with RMS have a poorer overall survival than pediatric patients, and survival varies considerably across histological subtypes. |
| format | Article |
| id | doaj-art-f01dfe902f6540f4a0f821d9a0bb42c1 |
| institution | Kabale University |
| issn | 2045-7634 |
| language | English |
| publishDate | 2018-08-01 |
| publisher | Wiley |
| record_format | Article |
| series | Cancer Medicine |
| spelling | doaj-art-f01dfe902f6540f4a0f821d9a0bb42c12024-12-20T13:15:45ZengWileyCancer Medicine2045-76342018-08-01784023403510.1002/cam4.1374Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide studyEmmanuelle Bompas0Loïc Campion1Antoine Italiano2Axel Le Cesne3Christine Chevreau4Nicolas Isambert5Maud Toulmonde6Olivier Mir7Isabelle Ray‐Coquard8Sophie Piperno‐Neumann9Esma Saada‐Bouzid10Maria Rios11Jean‐Emmanuel Kurtz12Corinne Delcambre13Pascale Dubray‐Longeras14Florence Duffaud15Marie Karanian16François Le Loarer17Patrick Soulié18Nicolas Penel19Jean‐Yves Blay20Institut de cancérologie de l'ouest – René Gauducheau Saint Herblain FranceInstitut de cancérologie de l'ouest – René Gauducheau Saint Herblain FranceInstitut Bergonié Bordeaux FranceInstitut Gustave Roussy Villejuif FranceInstitut Universitaire du Cancer Toulouse– Oncopole Toulouse FranceCentre Georges‐François Leclerc Dijon FranceInstitut Bergonié Bordeaux FranceInstitut Gustave Roussy Villejuif FranceCentre Léon Bérard Lyon FranceInstitut Curie Paris FranceCentre Antoine Lacassagne Nice FranceInstitut de Cancérologie de Lorraine Vandoeuvre‐les‐Nancy FranceHôpitaux universitaires de Strasbourg Strasbourg FranceCentre François Baclesse Caen FranceCentre Jean Perrin Clermont‐Ferrand FranceCHU de Marseille Marseille FranceCentre Léon Bérard Lyon FranceInstitut Bergonié Bordeaux FranceInstitut de cancérologie de l'ouest – Paul Papin Angers FranceCentre Oscar Lambret Lille FranceCentre Léon Bérard Lyon FranceAbstract Five‐year overall survival (OS) of localized RMS exceeds 70% in children (<18) but is very poor in adult patients. We analyzed the outcome and prognostic factors (PF) of a national series of adult patients with RMS in a large study. The study population consisted of two different cohorts: a retrospective cohort (157 adult patients treated in 13 reference centers between 05/1981 and 02/2010) and the prospective cohort (292 patients with RMS diagnosed and treated between 01/2010 and 12/2014 in France) included in the NetSarc database. A descriptive analysis of patients’ characteristics and prognostic factors was conducted on both series which were compared. In the retrospective series, histological subtypes were embryonal (E‐RMS) for 21% of patients, alveolar (A‐RMS) for 35% of patients, and “adult‐type” P‐RMS (pleomorphic, spindle cell RMS, not otherwise specified) (P) for 44% patients. This distribution significantly differed in the prospective cohort: A‐RMS: 18%; E‐RMS: 17%; and P‐RMS 65%. With a median follow‐up of 8.5 years, 5‐year OS for localized RMS and advanced RMS (with nodes and/or metastases) was 43% and 5%, respectively, (P < 0.0001), and median OS was 51, 33, and 16 months for E‐RMS, A‐RMS, and P‐RMS, respectively, in the retrospective cohort. The median OS was less than 40 months for the prospective nationwide cohort for the entire population. In a multivariate analysis of the retrospective study, independent prognostic factors for OS were A‐RMS, R0 resection, and adjuvant radiotherapy (RT). For localized RMS, age and use of pediatric chemotherapy (CT) regimen are independent prognostic factors. Adult patients with RMS have a poorer overall survival than pediatric patients, and survival varies considerably across histological subtypes.https://doi.org/10.1002/cam4.1374Adult cancerpediatricrhabdomyosarcoma |
| spellingShingle | Emmanuelle Bompas Loïc Campion Antoine Italiano Axel Le Cesne Christine Chevreau Nicolas Isambert Maud Toulmonde Olivier Mir Isabelle Ray‐Coquard Sophie Piperno‐Neumann Esma Saada‐Bouzid Maria Rios Jean‐Emmanuel Kurtz Corinne Delcambre Pascale Dubray‐Longeras Florence Duffaud Marie Karanian François Le Loarer Patrick Soulié Nicolas Penel Jean‐Yves Blay Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study Cancer Medicine Adult cancer pediatric rhabdomyosarcoma |
| title | Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study |
| title_full | Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study |
| title_fullStr | Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study |
| title_full_unstemmed | Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study |
| title_short | Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study |
| title_sort | outcome of 449 adult patients with rhabdomyosarcoma an observational ambispective nationwide study |
| topic | Adult cancer pediatric rhabdomyosarcoma |
| url | https://doi.org/10.1002/cam4.1374 |
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