PDF(Pubmed)
Abstract：
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.
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.
摘要：
背景：CIC重排肉瘤（CIC-RS）代表“尤因样”未分化小圆细胞肉瘤的最常见子集。这些肿瘤往往比尤因肉瘤更具侵袭性。此外，治疗策略可能因团队而异。这项回顾性研究的主要目的是描述其特征，治疗,和CIC-RS患者的结局包括在法国NETSARC+数据库中。
方法：从2008年10月至2021年3月登记了13个法国中心诊断为CIC-RS的儿科和成年患者。患者和肿瘤特征收集自国家网络NETSARC+数据库(http://netsarc。sarcomabcb.org)。CIC-RS诊断在病理和分子上得到了专家病理学家的集中审查。研究了两组患者：根据ESMO和/或EpSSG指南，作为经典尤因肉瘤（EwS队列）治疗的患者和作为高级软组织肉瘤（STS队列）治疗的患者。使用Kaplan-Meier方法计算存活率,并且使用对数秩检验来比较存活率。
结果：在79名患者中，男女性别比例为0.7,诊断时的中位年龄为27岁(范围2~87岁).中位随访时间为37个月，39例患者死于该疾病。诊断后的中位总生存期为18个月，两组之间没有显着差异（p=0.9）。然而，当专注于诊断时患有转移性疾病的患者（N=21），来自队列STS的所有患者均死于疾病,而来自队列EwS的一些患者仍然存活并且处于完全缓解.
结论：FSG的经验证实了无论化疗方案如何，CDS患者的积极临床过程。
方法：从2008年10月至2021年3月登记了13个法国中心诊断为CIC-RS的儿科和成年患者。患者和肿瘤特征收集自国家网络NETSARC+数据库(http://netsarc。sarcomabcb.org)。CIC-RS诊断在病理和分子上得到了专家病理学家的集中审查。研究了两组患者：根据ESMO和/或EpSSG指南，作为经典尤因肉瘤（EwS队列）治疗的患者和作为高级软组织肉瘤（STS队列）治疗的患者。使用Kaplan-Meier方法计算存活率,并且使用对数秩检验来比较存活率。
结果：在79名患者中，男女性别比例为0.7,诊断时的中位年龄为27岁(范围2~87岁).中位随访时间为37个月，39例患者死于该疾病。诊断后的中位总生存期为18个月，两组之间没有显着差异（p=0.9）。然而，当专注于诊断时患有转移性疾病的患者（N=21），来自队列STS的所有患者均死于疾病,而来自队列EwS的一些患者仍然存活并且处于完全缓解.
结论：FSG的经验证实了无论化疗方案如何，CDS患者的积极临床过程。
