Abstract：
Identifying prognostic factors and evaluating the impact of adjuvant chemotherapy in patients with sex cord stromal tumors (SCST) is crucial. In this study, we aimed to address these challenges.
We conducted a retrospective analysis of data from 13 centers of the French Rare malignant gynecological tumors (TMRG) network. We enrolled 469 adult patients with malignant SCST who received upfront surgery since 2011 to July 2015.
75% were diagnosed with adult Granulosa cell tumors, and 23% had another subtype. With a median follow-up of 6.4 years, 154 patients (33%) developed a first recurrence, 82 (17%) two recurrences, and 49 (10%) three recurrences. Adjuvant chemotherapy was administered in 14.7% of patients at initial diagnosis. In relapse, perioperative chemotherapy was administered in 58.5%, 28.2%, and 23.8% of patients, respectively, in the first, second, and third relapse. In the first-line therapy, age under 70 years, FIGO stage, and complete surgery were associated with longer progression-free survival (PFS). Chemotherapy had no impact on PFS in early-stage disease (FIGO I-II). The PFS was similar using BEP or other chemotherapy regimens (HR 0.88 [0.43; 1.81]) in the first-line therapy. In case of recurrence, PFS was statistically prolonged by complete surgery, but perioperative chemotherapy use did not impact PFS.
Chemotherapy use did not impact survival in the first-line or relapse setting in SCST. Only surgery and its quality demonstrated benefit for PFS in ovarian SCST in any lines of treatment.
We conducted a retrospective analysis of data from 13 centers of the French Rare malignant gynecological tumors (TMRG) network. We enrolled 469 adult patients with malignant SCST who received upfront surgery since 2011 to July 2015.
75% were diagnosed with adult Granulosa cell tumors, and 23% had another subtype. With a median follow-up of 6.4 years, 154 patients (33%) developed a first recurrence, 82 (17%) two recurrences, and 49 (10%) three recurrences. Adjuvant chemotherapy was administered in 14.7% of patients at initial diagnosis. In relapse, perioperative chemotherapy was administered in 58.5%, 28.2%, and 23.8% of patients, respectively, in the first, second, and third relapse. In the first-line therapy, age under 70 years, FIGO stage, and complete surgery were associated with longer progression-free survival (PFS). Chemotherapy had no impact on PFS in early-stage disease (FIGO I-II). The PFS was similar using BEP or other chemotherapy regimens (HR 0.88 [0.43; 1.81]) in the first-line therapy. In case of recurrence, PFS was statistically prolonged by complete surgery, but perioperative chemotherapy use did not impact PFS.
Chemotherapy use did not impact survival in the first-line or relapse setting in SCST. Only surgery and its quality demonstrated benefit for PFS in ovarian SCST in any lines of treatment.
摘要：
目的：确定预后因素并评估辅助化疗对性索间质瘤（SCST）患者的影响至关重要。在这项研究中,我们旨在应对这些挑战。
方法：我们对法国罕见妇科恶性肿瘤（TMRG）网络的13个中心的数据进行了回顾性分析。我们纳入了自2011年至2015年7月接受前期手术的469例恶性SCST成人患者。
结果：75%被诊断为成人颗粒细胞瘤，23%有另一种亚型。中位随访时间为6.4年，154名患者（33%）首次复发，82(17%)两次复发，和49（10％）三次复发。14.7%的患者在初次诊断时接受了辅助化疗。在复发中，围手术期化疗占58.5%，28.2%,23.8%的患者，分别,在第一,第二,第三次复发。在一线治疗中，年龄在70岁以下,FIGO阶段，和完整的手术与更长的无进展生存期（PFS）相关。化疗对早期疾病的PFS没有影响(FIGOI-II)。在一线治疗中，使用BEP或其他化疗方案的PFS相似（HR0.88[0.43;1.81]）。如果复发，完全手术后PFS在统计学上延长，但围手术期化疗的使用并不影响PFS.
结论：在SCST的一线或复发情况下，使用化疗不会影响生存率。在任何治疗方法中，只有手术及其质量证明了对卵巢SCSTPFS的益处。
方法：我们对法国罕见妇科恶性肿瘤（TMRG）网络的13个中心的数据进行了回顾性分析。我们纳入了自2011年至2015年7月接受前期手术的469例恶性SCST成人患者。
结果：75%被诊断为成人颗粒细胞瘤，23%有另一种亚型。中位随访时间为6.4年，154名患者（33%）首次复发，82(17%)两次复发，和49（10％）三次复发。14.7%的患者在初次诊断时接受了辅助化疗。在复发中，围手术期化疗占58.5%，28.2%,23.8%的患者，分别,在第一,第二,第三次复发。在一线治疗中，年龄在70岁以下,FIGO阶段，和完整的手术与更长的无进展生存期（PFS）相关。化疗对早期疾病的PFS没有影响(FIGOI-II)。在一线治疗中，使用BEP或其他化疗方案的PFS相似（HR0.88[0.43;1.81]）。如果复发，完全手术后PFS在统计学上延长，但围手术期化疗的使用并不影响PFS.
结论：在SCST的一线或复发情况下，使用化疗不会影响生存率。在任何治疗方法中，只有手术及其质量证明了对卵巢SCSTPFS的益处。
