Centre Léon Bérard, Lyon, France.
Gustave Roussy Cancer Campus, Villejuif, France.
Cancer Med. 2023 Apr;12(7):7801-7807. doi: 10.1002/cam4.5539. Epub 2022 Dec 20.
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.
CIC 重排肉瘤（CIC-RS）是“尤文样”未分化小圆细胞肉瘤中最常见的亚型。这些肿瘤比尤文肉瘤更具侵袭性。此外，治疗策略可能因团队而异。本回顾性研究的主要目的是描述纳入法国 NETSARC+数据库的 CIC-RS 患者的特征、治疗方法和结局。
从 2008 年 10 月至 2021 年 3 月，13 个法国中心的小儿和成人患者被纳入研究，这些患者的诊断均为 CIC-RS。患者和肿瘤特征从国家网络 NETSARC+数据库（http://netsarc.sarcomabcb.org）中收集。通过专家病理学家的中心审查，对 CIC-RS 进行病理和分子学确诊。研究了两组患者：根据 ESMO 和/或 EpSSG 指南，将其作为经典尤文肉瘤（EwS 组）治疗的患者，和将其作为高级软组织肉瘤（STS 组）治疗的患者。使用 Kaplan-Meier 法计算生存情况，并使用对数秩检验比较生存情况。
在 79 例患者中，男女比例为 0.7，诊断时的中位年龄为 27 岁（范围 2-87）。中位随访 37 个月后，39 例患者死于该疾病。从诊断开始的总生存中位数为 18 个月，两组之间无显著差异（p=0.9）。然而，当关注诊断时患有转移性疾病的患者（N=21）时，STS 组的所有患者均死于疾病，而一些 EwS 组的患者仍存活且处于完全缓解状态。
FSG 的经验证实了 CDS 患者无论化疗方案如何，其临床病程均具有侵袭性。
