Allison L Brodsky, Alejandra Flores Legarreta, Bryan M Fellman, Deanna Glassman, Jeffrey How, Veena Vuttaradhi, Anil K Sood, Lois Michelle Ramondetta, David Gershenson, Robert Tyler Hillman
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引用次数: 0
Abstract
Objectives: To evaluate survival outcomes among patients with adult-type granulosa cell tumors who have telomerase reverse transcriptase (TERT) promoter mutations.
Methods: This is a retrospective cohort study using the MD Anderson Rare Gynecologic Malignancy Registry. Patients with adult granulosa cell tumors who underwent molecular testing for TERT promoter and FOXL2 c.C402G mutations were included. We used descriptive statistics to compare demographic and clinical variables and estimated progression-free and overall survival with Kaplan-Meier curves. Cox proportional hazards regression and log-rank tests were employed for comparisons, with multivariable analyses adjusting for various factors.
Results: Among 70 patients, 28 (40%) had TERT+ tumors. The median age at diagnosis was 40 years (range 12-71) for TERT- patients and 46 years (range 25-76) for TERT+ patients. At diagnosis, 22 (63%) of 35 TERT- patients were stage I, 10 (29%) stage II, and 3 (9%) stage III, while in the TERT+ group, 17/23 (74%) were stage I, 3 (13%) stage II, and 3 (13%) stage II. Univariable analysis showed no difference in time from diagnosis to first recurrence (p=0.19) and from first recurrence to second recurrence (p=0.24) based on tumor TERT status. The median time from first to second recurrence in the TERT- group was 27.3 months (95% CI 14.1 to 40.0) and in the TERT+ group was 14.8 months (95% CI 8.1 to 21.0). There was no observed difference in overall survival between the groups (HR=0.53; 95% CI 0.19 to 1.45; p=0.21). Multivariable analysis adjusting for age at diagnosis, TERT promoter mutation status, systemic chemotherapy, and stage demonstrated a significant difference in progression-free survival based on TERT mutation status (HR=2.89; 95% CI 1.32 to 6.36).
Conclusions: After adjustment for covariates, patients with adult granulosa cell tumors and TERT+ tumors had shorter progression-free survival after first recurrence. TERT promoter mutations may identify a subset of patients with recurrent adult granulosa cell tumors and less favorable outcomes.
目的：评估端粒酶逆转录酶（TERT）启动子突变的成人型颗粒细胞瘤患者的生存结果：评估端粒酶逆转录酶（TERT）启动子突变的成人型颗粒细胞瘤患者的生存结果：这是一项利用 MD 安德森罕见妇科恶性肿瘤登记处进行的回顾性队列研究。研究纳入了接受TERT启动子和FOXL2 c.C402G突变分子检测的成人颗粒细胞瘤患者。我们使用描述性统计来比较人口统计学和临床变量，并通过 Kaplan-Meier 曲线来估计无进展生存期和总生存期。比较采用了 Cox 比例危险度回归和对数秩检验，并对各种因素进行了多变量分析：70名患者中，28人（40%）患有TERT+肿瘤。TERT-患者诊断时的中位年龄为40岁（12-71岁），TERT+患者为46岁（25-76岁）。确诊时，35 名 TERT- 患者中有 22 人（63%）为 I 期，10 人（29%）为 II 期，3 人（9%）为 III 期，而在 TERT+ 组中，17/23（74%）为 I 期，3 人（13%）为 II 期，3 人（13%）为 II 期。单变量分析显示，根据肿瘤TERT状态，从诊断到首次复发的时间（P=0.19）和从首次复发到第二次复发的时间（P=0.24）没有差异。TERT-组从首次复发到第二次复发的中位时间为27.3个月（95% CI 14.1-40.0），TERT+组为14.8个月（95% CI 8.1-21.0）。两组患者的总生存期无明显差异（HR=0.53；95% CI 0.19 至 1.45；P=0.21）。调整诊断年龄、TERT启动子突变状态、全身化疗和分期的多变量分析表明，基于TERT突变状态的无进展生存期存在显著差异（HR=2.89；95% CI 1.32至6.36）：经过协变量调整后，成人颗粒细胞肿瘤和TERT+肿瘤患者首次复发后的无进展生存期较短。TERT启动子突变可能会鉴别出复发性成人颗粒细胞瘤和预后较差的患者亚群。
期刊介绍：
The International Journal of Gynecological Cancer, the official journal of the International Gynecologic Cancer Society and the European Society of Gynaecological Oncology, is the primary educational and informational publication for topics relevant to detection, prevention, diagnosis, and treatment of gynecologic malignancies. IJGC emphasizes a multidisciplinary approach, and includes original research, reviews, and video articles. The audience consists of gynecologists, medical oncologists, radiation oncologists, radiologists, pathologists, and research scientists with a special interest in gynecological oncology.
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