Purpose

Patients with Von Hippel-Lindau disease often develop multifocal, metachronous renal cell carcinomas which require therapy. The purpose of this retrospective single-center study is to evaluate the outcomes of radiofrequency ablation (RFA) in the treatment of renal cell carcinomas in patients with Von Hippel-Lindau disease.

Materials and Methods

9 patients (4 male, 5 female, 47.9 ± 10.7 y/o) with Von Hippel-Lindau disease underwent 18 CT-guided percutaneous RFA procedures for the treatment 21 renal cell carcinomas (largest diameter: 32.9 ± 8.6 mm, cT1a: 16, cT1b: 5). Seven patients were previously treated either by partial or radical nephrectomy. Technical success, effectiveness, safety, progression-free survival, overall survival and tumor characteristics were analyzed.

Results

All RFA procedures were technically successful without major complications. There were 5 minor complications. No residual or recurrent tumor was seen in the ablation zone during a follow-up of 34.0 ± 18.1 months (0–58 months). No patient required dialysis during follow-up. One patient died after 63 months after the first treatment due to complications from a cerebellar hemangioblastoma. No endpoint was reached for overall or progression-free survival.

Conclusions

The results from this limited case series suggest that RFA of RCCs in patients with VHL is a safe and effective therapy, which can preserve sufficient renal function even after renal surgery.

中文翻译：

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Von Hippel-Lindau 病患者重复经皮射频消融 T1 肾细胞癌是安全的

目的

Von Hippel-Lindau 病患者经常发展为需要治疗的多灶性异时性肾细胞癌。这项回顾性单中心研究的目的是评估射频消融 (RFA) 治疗 Von Hippel-Lindau 病患者肾细胞癌的结果。

材料和方法

9 名 Von Hippel-Lindau 病患者（4 名男性，5 名女性，47.9 ± 10.7 y/o）接受了 18 次 CT 引导下经皮 RFA 手术治疗 21 例肾细胞癌（最大直径：32.9 ± 8.6 mm，cT1a：16， cT1b：5）。七名患者先前接受过部分或根治性肾切除术治疗。分析了技术成功、有效性、安全性、无进展生存期、总生存期和肿瘤特征。

结果

所有 RFA 程序在技术上都是成功的，没有出现重大并发症。有5个轻微并发症。在 34.0 ± 18.1 个月（0-58 个月）的随访期间，消融区未发现残留或复发的肿瘤。随访期间没有患者需要透析。一名患者在第一次治疗后 63 个月后死于小脑血管母细胞瘤并发症。没有达到总生存期或无进展生存期的终点。

结论

该有限病例系列的结果表明，对 VHL 患者 RCC 进行 RFA 是一种安全有效的治疗方法，即使在肾脏手术后也可以保持足够的肾功能。