Patterns of care and outcome of CIC-rearranged sarcoma patients: A nationwide study of the French sarcoma group.
Adult
Humans
Male
Female
Child
Child, Preschool
Adolescent
Young Adult
Middle Aged
Aged
Aged, 80 and over
Sarcoma, Ewing
/ genetics
Retrospective Studies
Sarcoma, Small Cell
/ diagnosis
Sarcoma
/ epidemiology
Bone Neoplasms
/ epidemiology
Soft Tissue Neoplasms
/ genetics
Skin Neoplasms
Death
Oncogene Proteins, Fusion
Biomarkers, Tumor
CIC-rearranged sarcomas
CIC::DUX4 sarcomas
Ewing-like sarcoma.
Ultra-rare sarcoma
Undifferentiated round cell sarcoma
Journal
Cancer medicine
ISSN: 2045-7634
Titre abrégé: Cancer Med
Pays: United States
ID NLM: 101595310
Informations de publication
Date de publication:
04 2023
04 2023
Historique:
revised:
04
11
2022
received:
19
09
2022
accepted:
29
11
2022
medline:
1
5
2023
pubmed:
21
12
2022
entrez:
20
12
2022
Statut:
ppublish
Résumé
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. 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. 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. FSG experience confirms the aggressive clinical course of CDS patients regardless of chemotherapy regimen.
Sections du résumé
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.
Identifiants
pubmed: 36537582
doi: 10.1002/cam4.5539
pmc: PMC10134374
doi:
Substances chimiques
Oncogene Proteins, Fusion
0
Biomarkers, Tumor
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
7801-7807Informations de copyright
© 2022 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.
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