von Hippel Lindau 病患者肾移植的结果和适应证。
Outcomes of and indications for renal transplantation in patients with von Hippel Lindau disease.
机构信息
Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD.
Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD.
出版信息
Urol Oncol. 2023 Dec;41(12):487.e1-487.e6. doi: 10.1016/j.urolonc.2023.09.003. Epub 2023 Sep 29.
INTRODUCTION AND OBJECTIVE
Von Hippel-Lindau (VHL) is a hereditary cancer syndrome characterized by bilateral, multifocal renal masses. The cumulative impact of extirpative surgery can depreciate renal function and render patients anephric. In the larger end-stage renal disease population, renal transplant offers both excellent quality of life and functional renal replacement. This case control study aims to examine and compare oncologic and functional outcomes of patients who have undergone renal transplant as renal replacement therapy (RRT) to those who remain anephric.
METHODS
Patient charts were retrospectively reviewed of patients with germline testing confirmed VHL between 1980 and 2022 for transplant, all prior surgical history (within and outside the NCI), renal function and graft outcomes. Overall survival (OS) was determined from years after radical nephrectomy, and graft time was defined as years of graft function from initial transplant until failure or patient death. Graft survival was determined as time between transplant(s) to last follow up. Kaplan-Meier analysis was conducted to compare graft times of anephric VHL patients to those with transplanted kidneys.
RESULTS
A total of 23 VHLD patients were identified as either anephric or candidates for transplant. Out of this cohort, 11 total VHLD received 12 total kidney grafts. Median wait time from nephrectomy to transplant was 22.6 months (IQR: 1.02-40.25 months). Median age at transplant was 32 years (IQR: 23-54 years). OS at 5 and 10 years of anephric patients who did not receive a transplant was 33% and 16.7%, respectively. OS rates of the transplant cohort at 10, 15, and 20 years were 91%, 78%, and 58% years, respectively. Median graft time was 161 months (IQR: 56-214 months). Graft survival at 10, 15, and 20 years was 69.8%, 69.8%, and 26.2%, respectively.
CONCLUSIONS
We demonstrate that transplant recipients have decreased mortality with no difference in cancer recurrence compared to those who do not receive renal transplant for RRT. This data can aid in informing providers of the optimal window for early RRT planning in VHL, while also improving patient counseling.
简介和目的
希佩尔-林道综合征（VHL）是一种遗传性癌症综合征，其特征为双侧、多灶性肾肿瘤。根治性手术的累积影响可能会降低肾功能，导致患者无肾功能。在更大的终末期肾病患者群体中，肾移植既能提供出色的生活质量，也能提供功能性肾功能替代。本病例对照研究旨在检查和比较接受肾移植作为肾脏替代治疗（RRT）的患者与保持无肾功能的患者的肿瘤学和功能结果。
方法
对 1980 年至 2022 年间接受过基因检测证实的 VHL 患者的病历进行回顾性研究，所有患者均接受过手术治疗（包括 NCI 内外），并对肾功能和移植物结果进行评估。总生存期（OS）从根治性肾切除术开始计算，移植物时间定义为从初始移植到移植物衰竭或患者死亡的移植功能年数。移植物存活率是从移植（次）到最后一次随访的时间。采用 Kaplan-Meier 分析比较无功能 VHL 患者与移植肾患者的移植物时间。
结果
共确定了 23 例 VHLD 患者为无功能或适合接受移植。在这一队列中，共有 11 例 VHLD 患者接受了 12 次肾脏移植。从肾切除术到移植的中位等待时间为 22.6 个月（IQR：1.02-40.25 个月）。移植时的中位年龄为 32 岁（IQR：23-54 岁）。未接受移植的无功能患者的 5 年和 10 年总生存率分别为 33%和 16.7%。移植组在 10、15 和 20 年的生存率分别为 91%、78%和 58%。中位移植物时间为 161 个月（IQR：56-214 个月）。10、15 和 20 年的移植物存活率分别为 69.8%、69.8%和 26.2%。
结论
我们表明，与未接受肾移植作为 RRT 的患者相比，移植受者的死亡率降低，且癌症复发率无差异。该数据可以为 VHL 患者早期 RRT 计划的最佳时机提供信息，并改善患者咨询。
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