ICO Centre René Gauducheau, Oncology, Nantes, France.
ICO Centre René Gauducheau, Oncology, Nantes, France; CNRS, Inserm, CRCINA, Nantes, France.
ESMO Open. 2022 Apr;7(2):100402. doi: 10.1016/j.esmoop.2022.100402. Epub 2022 Feb 21.
Synovial sarcoma (SS) occurs in both adult and pediatric patients. The primary aim of this study is to describe the outcomes, prognostic factors, and treatment of patients with metastatic SS within a nationwide cohort.
All pediatric and adult patients with metastatic SS are registered in the French Sarcoma Group database. Data were collected from the national database https://conticabase.sarcomabcb.org/ up to March 2020. Descriptive and comparative analyses were conducted using SAS 9.4 and Stata Special Edition 16.1 software.
Between January 1981 and December 2019, 417 patients with metastatic SS from 17 French sarcoma centers were included, including 64 (15.3%) under the age of 26 years. Median age was 42.5 years (range 9-87 years). The metastases were synchronous (cohort 1) or metachronous (cohort 2) in 18.9% (N = 79) and 81.1% (N = 338) patients, respectively. Median overall survival (OS) from the date of metastasis was 22.3 months (95% confidence interval 19.7-24.1 months). First-line chemotherapy without ifosfamide and/or doxorubicin was unfavorable for progression-free survival and OS (P < 0.001). Concerning cohort 1, young age, surgery of the primary tumor, and single metastatic site were independent favorable prognostic factors for OS. In cohort 2, surgery within an expert French Sarcoma Group center, absence of chemotherapy in the perioperative setting, the lungs as a single metastatic site, time to first metastasis >12 months, local therapy, and ifosfamide in the first metastatic line were independent favorable prognostic factors.
The outcome of patients with metastatic SS is influenced by local treatment, management in reference centers, and cytotoxic treatments given in the perioperative and metastatic setting.
滑膜肉瘤（SS）发生于成人和儿童患者。本研究的主要目的是在全国性队列中描述转移性 SS 患者的结局、预后因素和治疗方法。
所有转移性 SS 患儿和成人患者均在法国肉瘤组数据库中登记。数据来自国家数据库 https://conticabase.sarcomabcb.org/，截至 2020 年 3 月。采用 SAS 9.4 和 Stata Special Edition 16.1 软件进行描述性和比较性分析。
1981 年 1 月至 2019 年 12 月，17 个法国肉瘤中心的 417 例转移性 SS 患者被纳入研究，其中 64 例（15.3%）年龄小于 26 岁。中位年龄为 42.5 岁（范围 9-87 岁）。转移是同步（队列 1）或异时（队列 2）的分别占 18.9%（N=79）和 81.1%（N=338）。从转移日期开始的中位总生存期（OS）为 22.3 个月（95%置信区间 19.7-24.1 个月）。不包含异环磷酰胺和/或阿霉素的一线化疗对无进展生存期和 OS 不利（P<0.001）。对于队列 1，年轻年龄、原发肿瘤手术和单一转移部位是 OS 的独立有利预后因素。在队列 2 中，在法国肉瘤组中心进行手术、围手术期无化疗、肺部为单一转移部位、首次转移时间>12 个月、局部治疗和一线使用异环磷酰胺是独立的有利预后因素。
转移性 SS 患者的预后受局部治疗、在参考中心的治疗管理以及围手术期和转移期使用细胞毒药物治疗的影响。
