Patterns of care and outcome of CIC‐rearranged sarcoma patients: A nationwide study of the French sarcoma group
Abstract Background CIC‐rearranged sarcomas (CIC‐RS) represent the most frequent subset of “Ewing‐like” undifferentiated small round cell sarcomas. These tumors tend to be more aggressive than Ewing sarcomas. Moreover, treatment strategy can differ according to teams. The primary aim of this retrosp...
| Main Authors: | , , , , , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-04-01
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| Series: | Cancer Medicine |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/cam4.5539 |
| _version_ | 1797838362530283520 |
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| author | Mehdi Brahmi Nathalie Gaspar Justine Gantzer Maud Toulmonde Pascaline Boudou‐Rouquette emmanuelle Bompas Nelly Firmin Thibaud Valentin Mathilde Cancel Florence Duffaud Francois Bertucci Christophe Perrin Armelle Dufresne Perrine Marec‐Bérard Myriam Jean‐Denis Isabelle Ray‐Coquard Francois Le Loarer Gaille Pierron Franck Tirode Jean‐Yves Blay Sarah Watson |
| author_facet | Mehdi Brahmi Nathalie Gaspar Justine Gantzer Maud Toulmonde Pascaline Boudou‐Rouquette emmanuelle Bompas Nelly Firmin Thibaud Valentin Mathilde Cancel Florence Duffaud Francois Bertucci Christophe Perrin Armelle Dufresne Perrine Marec‐Bérard Myriam Jean‐Denis Isabelle Ray‐Coquard Francois Le Loarer Gaille Pierron Franck Tirode Jean‐Yves Blay Sarah Watson |
| author_sort | Mehdi Brahmi |
| collection | DOAJ |
| description | Abstract Background CIC‐rearranged sarcomas (CIC‐RS) represent the most frequent subset of “Ewing‐like” undifferentiated small round cell sarcomas. These tumors tend to be more aggressive than Ewing sarcomas. Moreover, treatment strategy can differ according to teams. The primary aim of this retrospective study was to describe the characteristics, treatments, and outcome for patients with CIC‐RS included in the French NETSARC+ database. Methods Pediatric and adult patients from 13 French centers with a diagnosis of CIC‐RS were registered from October 2008 to March 2021. Patients and tumors characteristics were collected from the national network NETSARC+ database (http://netsarc.sarcomabcb.org). CIC‐RS diagnosis was pathologically and molecularly confirmed with a central review by expert pathologists. Two groups of patients were studied: those treated as classical Ewing sarcomas (cohort EwS) and those treated as high‐grade soft tissue sarcomas (cohort STS) according to ESMO and/or EpSSG guidelines. Survival was calculated using the Kaplan–Meier method and the log‐rank test was used to compare survival. Results Among 79 patients, the male/female sex ratio was 0.7 and the median age at diagnosis was 27 years (range 2–87). With a median follow‐up of 37 months, 39 patients died of the disease. Median overall survival from diagnosis was 18 months, with no significant difference between both cohorts (p = 0.9). Nevertheless, when focusing on patients with metastatic disease at diagnosis (N = 21), all patients from cohort STS died of disease while some patients from cohort EwS were still alive and in complete remission. Conclusion FSG experience confirms the aggressive clinical course of CDS patients regardless of chemotherapy regimen. |
| first_indexed | 2024-04-09T15:40:39Z |
| format | Article |
| id | doaj.art-3384f7a641a44b0195ea12b9f77ba553 |
| institution | Directory Open Access Journal |
| issn | 2045-7634 |
| language | English |
| last_indexed | 2024-04-09T15:40:39Z |
| publishDate | 2023-04-01 |
| publisher | Wiley |
| record_format | Article |
| series | Cancer Medicine |
| spelling | doaj.art-3384f7a641a44b0195ea12b9f77ba5532023-04-27T10:12:43ZengWileyCancer Medicine2045-76342023-04-011277801780710.1002/cam4.5539Patterns of care and outcome of CIC‐rearranged sarcoma patients: A nationwide study of the French sarcoma groupMehdi Brahmi0Nathalie Gaspar1Justine Gantzer2Maud Toulmonde3Pascaline Boudou‐Rouquette4emmanuelle Bompas5Nelly Firmin6Thibaud Valentin7Mathilde Cancel8Florence Duffaud9Francois Bertucci10Christophe Perrin11Armelle Dufresne12Perrine Marec‐Bérard13Myriam Jean‐Denis14Isabelle Ray‐Coquard15Francois Le Loarer16Gaille Pierron17Franck Tirode18Jean‐Yves Blay19Sarah Watson20Centre Léon Bérard Lyon FranceGustave Roussy Cancer Campus Villejuif FranceICANS Strabourg FranceInstitut Bergonié Bordeaux FranceHopital Cochin Saint Vincent de Paul Paris FranceICO Angers FranceICM Montpellier FranceInstitut Claudius Regaud Toulouse FranceCHU Bretonneau Tours FranceCHU Timone Marseille FranceInstitut Paoli‐Calmettes Marseille FranceCentre Eugène Marquis Rennes FranceCentre Léon Bérard Lyon FranceCentre Léon Bérard Lyon FranceCentre Léon Bérard Lyon FranceCentre Léon Bérard Lyon FranceInstitut Bergonié Bordeaux FranceInstitut Curie Paris FranceCentre Léon Bérard Lyon FranceCentre Léon Bérard Lyon FranceInstitut Curie Paris FranceAbstract Background CIC‐rearranged sarcomas (CIC‐RS) represent the most frequent subset of “Ewing‐like” undifferentiated small round cell sarcomas. These tumors tend to be more aggressive than Ewing sarcomas. Moreover, treatment strategy can differ according to teams. The primary aim of this retrospective study was to describe the characteristics, treatments, and outcome for patients with CIC‐RS included in the French NETSARC+ database. Methods Pediatric and adult patients from 13 French centers with a diagnosis of CIC‐RS were registered from October 2008 to March 2021. Patients and tumors characteristics were collected from the national network NETSARC+ database (http://netsarc.sarcomabcb.org). CIC‐RS diagnosis was pathologically and molecularly confirmed with a central review by expert pathologists. Two groups of patients were studied: those treated as classical Ewing sarcomas (cohort EwS) and those treated as high‐grade soft tissue sarcomas (cohort STS) according to ESMO and/or EpSSG guidelines. Survival was calculated using the Kaplan–Meier method and the log‐rank test was used to compare survival. Results Among 79 patients, the male/female sex ratio was 0.7 and the median age at diagnosis was 27 years (range 2–87). With a median follow‐up of 37 months, 39 patients died of the disease. Median overall survival from diagnosis was 18 months, with no significant difference between both cohorts (p = 0.9). Nevertheless, when focusing on patients with metastatic disease at diagnosis (N = 21), all patients from cohort STS died of disease while some patients from cohort EwS were still alive and in complete remission. Conclusion FSG experience confirms the aggressive clinical course of CDS patients regardless of chemotherapy regimen.https://doi.org/10.1002/cam4.5539CIC::DUX4 sarcomasCIC‐rearranged sarcomasUndifferentiated round cell sarcomaUltra‐rare sarcomaEwing‐like sarcoma. |
| spellingShingle | Mehdi Brahmi Nathalie Gaspar Justine Gantzer Maud Toulmonde Pascaline Boudou‐Rouquette emmanuelle Bompas Nelly Firmin Thibaud Valentin Mathilde Cancel Florence Duffaud Francois Bertucci Christophe Perrin Armelle Dufresne Perrine Marec‐Bérard Myriam Jean‐Denis Isabelle Ray‐Coquard Francois Le Loarer Gaille Pierron Franck Tirode Jean‐Yves Blay Sarah Watson Patterns of care and outcome of CIC‐rearranged sarcoma patients: A nationwide study of the French sarcoma group Cancer Medicine CIC::DUX4 sarcomas CIC‐rearranged sarcomas Undifferentiated round cell sarcoma Ultra‐rare sarcoma Ewing‐like sarcoma. |
| title | Patterns of care and outcome of CIC‐rearranged sarcoma patients: A nationwide study of the French sarcoma group |
| title_full | Patterns of care and outcome of CIC‐rearranged sarcoma patients: A nationwide study of the French sarcoma group |
| title_fullStr | Patterns of care and outcome of CIC‐rearranged sarcoma patients: A nationwide study of the French sarcoma group |
| title_full_unstemmed | Patterns of care and outcome of CIC‐rearranged sarcoma patients: A nationwide study of the French sarcoma group |
| title_short | Patterns of care and outcome of CIC‐rearranged sarcoma patients: A nationwide study of the French sarcoma group |
| title_sort | patterns of care and outcome of cic rearranged sarcoma patients a nationwide study of the french sarcoma group |
| topic | CIC::DUX4 sarcomas CIC‐rearranged sarcomas Undifferentiated round cell sarcoma Ultra‐rare sarcoma Ewing‐like sarcoma. |
| url | https://doi.org/10.1002/cam4.5539 |
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