背景:罕见的泌尿生殖系统肿瘤缺乏随机和观察性数据。我们旨在通过Meet-URO23/I-RARE数据库描述集合管癌(CDC)患者的临床特征和结果。
方法:我们在Meet-URO网络中进行了一项多中心的回顾性前瞻性研究,从2021年3月(从2011年回顾性)到2023年3月招募患者。主要目的是描述CDC患者的临床特征,次要目标是根据无复发生存期(RFS)评估肿瘤结局,无进展生存期(PFS),总生存期(OS)和治疗客观缓解率(ORR)。
结果:纳入37例CDC患者。四名患者只接受了手术,33人接受了一线全身治疗。中位OS为22.1个月(95%CI,8.9-31.9)。局部疾病患者发病时的中位RFS(n=30)为3.7个月(95%CI,1.9-12.8),一线治疗的中位PFS为3.3个月(95%CI,2.7-9.9),ORR为27%。女性性别和良好的工作状态(PS)与较长的PFS(分别为P=.072和P<.01)和OS(分别为P=.030和P=.141)相关。
结论:CDC患者预后不佳,从现有的治疗中获益很少。女性和良好的PS似乎与更好的预后有关。
BACKGROUND: Rare genitourinary tumors are lacking of randomized and observational data. We aimed to describe the clinical characteristics and outcomes of patients with collecting duct carcinoma (CDC) through the Meet-URO 23/I-
RARE database.
METHODS: We performed a multicentric retrospective-prospective study within the Meet-URO network, enrolling patients from March 2021 (retrospectively up from 2011) until March 2023. The primary objective was to describe the clinical characteristics of patients with CDC, the secondary objectives were to assess the oncological outcomes in terms of relapse-free survival (RFS), progression-free survival (PFS), overall survival (OS) and objective response rate (ORR) to treatment.
RESULTS: 37 patients with CDC were enrolled. Four patients underwent only surgery, 33 received first-line systemic therapy. Median OS was 22.1 months (95% CI, 8.9-31.9). Median RFS for patients with localized disease at onset (n = 30) was 3.7 months (95% CI, 1.9-12.8), median PFS for first-line treatment was 3.3 months (95% CI, 2.7-9.9), with an ORR of 27%. Female sex and good performance status (PS) were associated with longer PFS (P = .072 and P < .01, respectively) and OS (P = .030 and P = .141, respectively).
CONCLUSIONS: Patients with CDC had dismal prognosis, with scarce benefit from the available treatments. Female sex and good PS seemed to be associated with better prognosis.