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复发性子宫平滑肌肉瘤患者的临床特征和治疗结局。

Clinical characteristics and treatment outcomes of women with recurrent uterine leiomyosarcoma.

机构信息

Department of Gynecologic Oncology, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 17 # Panjiayuannanli, Chaoyang District, Beijing, 100021, China.

Department of Pathology, National Cancer Center/ National Clinical Research Center for Cancer/ Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100021, China.

出版信息

Orphanet J Rare Dis. 2024 Oct 25;19(1):395. doi: 10.1186/s13023-024-03415-3.

Abstract

OBJECTIVE

To determine the clinical characteristics and treatment outcomes of women with recurrent uterine leiomyosarcoma (uLMS).

METHODS

We conducted a retrospective cohort study to evaluate the clinical characteristics and survival of women with recurrent uLMS and identify prognostic factors.

RESULTS

Overall, 71 patients with first recurrence of uLMS were included in our study. 19 patients (26.8%) received systemic therapy and 52 patients (73.2%) received secondary cytoreductive surgery (SCS). In SCS subgroup (n = 52), a complete resection with no residual disease was performed in 47 patients (90.4%). 38.5% (20/52) patients received non-reproductive organ surgeries. 10 (19.2%) patients had received thoracic surgery because of lung-only recurrence. Bowel, bladder surgery was performed in 8 (15.4%), 3 (5.8%) patients, respectively. 1 (1.9%) patient had received liver surgery. The median follow-up duration was 38.7 months (range: 2.7-317.6 months). 41 (57.7%) patients died during follow-up. 5-year OS for the entire cohort was 52.9%. Patients experienced first recurrence after initial diagnoses within 12 months (n = 24) had a worse 5-year OS than those after 12 months (n = 47) (17.0% vs. 69.1%, P < 0.001). 5-year OS for the SCS and non-SCS subgroup was 62.0% and 28.0%, respectively (P < 0.001). Multivariate analysis showed time to fist recurrence within 12 months (HR = 4.60, 95% CI: 1.49-14.4, P = 0.008) was an independent predictor of decreased 5-year OS in SCS subgroup.

CONCLUSIONS

SCS is an important treatment choice for recurrent uLMS and seems to have benefited patients. Time to fist recurrence within 12 months is an independent predictor of decreased 5-year OS in SCS subgroup.

摘要

目的

确定复发性子宫平滑肌肉瘤(uLMS)患者的临床特征和治疗结局。

方法

我们进行了一项回顾性队列研究,以评估复发性 uLMS 患者的临床特征和生存情况,并确定预后因素。

结果

总体而言,我们的研究纳入了 71 例首次 uLMS 复发患者。19 例(26.8%)患者接受了系统治疗,52 例(73.2%)患者接受了二次细胞减灭术(SCS)。在 SCS 亚组(n=52)中,47 例(90.4%)患者行完全切除且无残留病灶。38.5%(20/52)患者接受了非生殖器官手术。10 例(19.2%)患者因单纯肺转移而接受了胸部手术。8 例(15.4%)、3 例(5.8%)患者分别接受了肠、膀胱手术。1 例(1.9%)患者接受了肝脏手术。中位随访时间为 38.7 个月(范围:2.7-317.6 个月)。41 例(57.7%)患者在随访期间死亡。全队列的 5 年 OS 为 52.9%。初始诊断后 12 个月内(n=24)发生首次复发的患者,5 年 OS 较初始诊断后 12 个月后(n=47)的患者更差(17.0%比 69.1%,P<0.001)。SCS 和非 SCS 亚组的 5 年 OS 分别为 62.0%和 28.0%(P<0.001)。多因素分析显示,12 个月内首次复发时间(HR=4.60,95%CI:1.49-14.4,P=0.008)是 SCS 亚组 5 年 OS 降低的独立预测因素。

结论

SCS 是复发性 uLMS 的重要治疗选择,似乎使患者受益。12 个月内首次复发时间是 SCS 亚组 5 年 OS 降低的独立预测因素。

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