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Mini oral session - Sarcoma

1524MO - Patterns of care and outcomes of 64 CIC-rearranged sarcoma: A retrospective multicentre case-series within the French Sarcoma Group (FSG)


17 Sep 2021


Mini oral session - Sarcoma


Brahmi Mehdi


Annals of Oncology (2021) 32 (suppl_5): S1111-S1128. 10.1016/annonc/annonc712


B. Mehdi1, N. Gaspar2, J. Gantzer3, M. Toulmonde4, P. Boudou Rouquette5, E. Bompas6, N. Firmin7, T. Valentin8, M. Cancel9, F. Duffaud10, F. Bertucci11, A. Brunot12, A. Dufresne13, P. Marec Berard14, M. Jean-Denis1, I.L. Ray-Coquard15, F. le Loarer16, F. Tirode17, J. Blay18, S. Watson19

Author affiliations

  • 1 Medical Oncology, Centre Léon Bérard, 69008 - Lyon/FR
  • 2 Child & Adolescent Oncology Dept., Institut Gustave Roussy, 94805 - Villejuif, Cedex/FR
  • 3 Medical Oncology Department, C.H.U. Strasbourg-Nouvel Hopital Civil, 67000 - Strasbourg/FR
  • 4 Medical Oncology, Institut Bergonie, 33076 - Bordeaux/FR
  • 5 Oncology, Hopital Cochin - Site Port-Royal AP-HP, 75014 - Paris/FR
  • 6 Oncology, ICO Institut de Cancerologie de l'Ouest René Gauducheau, 44805 - Saint-Herblain/FR
  • 7 Institut Du Cancer De Montpellier, Institut du Cancer de Montpellier, Montpellier/FR
  • 8 Institut Claudius Regaud, Institut Claudius Regaud, Toulouse/FR
  • 9 Medical Oncology, Pôle Santé Léonard de Vinci, 37170 - Chambray-lès-Tours/FR
  • 10 Aphp Marseilles, CHU La Timone Adultes, 13385 - Marseille/FR
  • 11 Medecine, Institut Paoli Calmettes, 13009 - Marseille/FR
  • 12 Medical Oncology, Centre Eugene - Marquis, 35042 - Rennes/FR
  • 13 Medical Oncology, Centre Leon Berard, 69008 - Lyon/FR
  • 14 Paediatric Department, Hematology and Oncology Pediatric Institute, 69008 - Lyon/FR
  • 15 Medical Oncology Department, Centre Léon Bérard, 69008 - Lyon/FR
  • 16 Department Of Biopathology, Institut Bergonie, 33076 - Bordeaux/FR
  • 17 Cancer Research Center Of Lyon, Centre Léon Bérard, 69008 - Lyon/FR
  • 18 Medicine Department, Centre Léon Bérard, 69008 - Lyon/FR
  • 19 26 Rue D'ulm, Institut Curie, 75005 - Paris/FR


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Abstract 1524MO


CIC-DUX4 sarcoma (CDS) represent the second most prevalent subset of high grade round cell sarcomas after Ewing sarcomas. The natural history might be diverse and probably underscores a heterogeneous group. Moreover, treatment strategy can differ according to institutions. The main goals of this retrospective study was to describe characteristics, treatments and outcome for patients with CDS included in the French NETSARC database.


Pediatric and adult patients from 11 French centers with a diagnosis of CDS were registered from October 2008 to March 2021. Patients and tumors characteristics were collected from the national network NETSARC+ database. CDS diagnosis was histologically and molecularly confirmed with a central review by expert pathologists. Two groups of patients were studied: those treated similarly to classical Ewing sarcomas especially with multi-agent chemotherapy in neoadjuvant and adjuvant setting (cohort 1) and those treated as high-grade soft tissue sarcomas according to ESMO and/or EpSSG guidelines (cohort 2).


Among the 64 patients with confirmed CDS, the sex ratio was 0.9 and the median age at diagnosis was 25 years (range 11-87) including 19 patients < 18. Three patients (5%) had a previous cancer history. Population and tumor characteristics were not statistically different between cohort 1 (N=38, 60%) and cohort 2 (N=26, 40%). With a median follow-up of 37 months (range 3-252), 32 patients (50%) died of the disease. Median overall survival from diagnosis was 4 years, with no significant difference between both cohorts (Log-rank test, P=0.99). Nevertheless, when focusing on patients with metastatic disease at diagnosis (N=18), all patients from cohort 2 (N=6) died due to progressive disease while 7 patients from cohort 1 are still alive and in complete remission, resulting in a significantly better OS (Log-rank test, P<0.05).


FSG experience confirms the aggressive clinical course of CDS patients regardless of chemotherapy regimen, even if this study suggests that metastatic patients should not benefit from a therapeutic de-escalation. Molecular studies are urgently needed to improve understanding and treatment of this orphan disease.

Clinical trial identification

Editorial acknowledgement

Legal entity responsible for the study

The authors.


Has not received any funding.


All authors have declared no conflicts of interest.

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