Unit of Urology, University Vita-Salute, IRCCS San Raffaele Scientific Institute, Via Olgettina 60, 20132, Milan, Italy.
Division of Experimental Oncology, URI, Urological Research Institute, IRCCS San Raffaele Scientific Institute, Milan, Italy.
World J Urol. 2021 Aug;39(8):2969-2975. doi: 10.1007/s00345-020-03574-5. Epub 2021 Jan 8.
To investigate the natural history and follow-up after kidney tumor treatment of Von Hippel-Lindau (VHL) patients.
A multi-institutional European consortium of patients with VHL syndrome included 96 non-metastatic patients treated at 9 urological departments (1987-2018). Descriptive and survival analyses were performed.
Median age at VHL diagnosis was 34 years (IQR 25-43). Two patients (2.1%) showed only renal manifestations at VHL diagnosis. Concomitant involvement of Central Nervous System (CNS) vs. pancreas vs. eyes vs. adrenal gland vs. others were present in 60.4 vs. 68.7 vs. 30.2 vs. 15.6 vs. 15.6% of patients, respectively. 45% of patients had both CNS and pancreatic diseases alongside kidney. The median interval between VHL diagnosis and renal cancer treatment resulted 79 months (IQR 0-132), and median index tumor size leading to treatment was 35.5 mm (IQR 28-60). Of resected malignant tumours, 73% were low grade. Of high-grade tumors, 61.1% were large > 4 cm. With a median follow-up of 8 years, clinical renal progression rate was 11.7% and 29.3% at 5 and 10 years, respectively. Overall mortality was 4% and 7.5% at 5 and 10 years, respectively. During the follow-up, 50% of patients did not receive a second active renal treatment. Finally, 25.3% of patients had CKD at last follow-up.
Mean period between VHL diagnosis and renal cancer detection is roughly three years, with significant variability. Although, most renal tumors are small low-grade, clinical progression and mortality are not negligible. Moreover, kidney function represents a key issue in VHL patients.
研究 von Hippel-Lindau(VHL) 患者肾肿瘤治疗后的自然病史和随访情况。
一个由欧洲多家机构组成的 VHL 综合征患者联盟，纳入了 9 个泌尿科部门（1987-2018 年）治疗的 96 例非转移性患者。进行了描述性和生存分析。
VHL 诊断时的中位年龄为 34 岁（IQR 25-43）。两名患者（2.1%）在 VHL 诊断时仅表现为肾脏表现。同时累及中枢神经系统（CNS）、胰腺、眼睛、肾上腺和其他部位的患者分别占 60.4%、68.7%、30.2%、15.6%和 15.6%。45%的患者同时患有 CNS 和胰腺疾病以及肾脏疾病。VHL 诊断和肾肿瘤治疗之间的中位间隔时间为 79 个月（IQR 0-132），导致治疗的中位指数肿瘤大小为 35.5 毫米（IQR 28-60）。切除的恶性肿瘤中，73%为低级别。高级别肿瘤中，61.1%的肿瘤直径大于 4 厘米。中位随访 8 年后，临床肾进展率分别为 11.7%和 29.3%，5 年和 10 年分别为 4%和 7.5%。随访期间，50%的患者未接受第二次主动肾脏治疗。最后，25.3%的患者在最后一次随访时患有 CKD。
VHL 诊断和肾肿瘤发现之间的平均时间约为三年，但存在很大的差异。尽管大多数肾肿瘤较小且为低级别，但临床进展和死亡率不容忽视。此外，肾功能是 VHL 患者的一个关键问题。
