Von Hipple Lindau患者多发性肾肿瘤切除术。逆行/经腹腔联合入路合并体内低温

V. Tubau, J. Bauza, E. Pieras, X. Brugarolas, P. Pizá
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摘要

目的与简介:探讨经腹膜和后腹膜腹腔镜联合入路治疗Von hipplel - lindau (VHL)多发肾肿瘤的可行性。VHL是一种常染色体显性遗传综合征,其特点是许多器官的良性和恶性肿瘤和囊肿的高发。肾细胞癌是最常见的死亡原因之一(1)。手术方法通常是复杂的,因为它的多样性,并且由于未来复发的风险,需要最大限度地保留肾功能(2,3)。在这些病例中,体内肾低温可能有助于防止永久性肾功能丧失(4)。材料和方法:40岁男性,监测双侧多个肾脏肿块。家族史包括影响其母亲和妹妹的VHL综合征。既往病史包括VHL综合征合并多发性小脑和髓质血管母细胞瘤、胰腺囊腺瘤和双侧肾肿瘤,这些肿瘤在随访期间明显增大。患者计划行腹腔镜多重部分肾切除术。选择TP + RP联合体外低温治疗。结果:6例右肾肿瘤全部切除。手术时间240 min,冷缺血时间50 min,平均肾温23.7℃。失血几乎可以忽略不计。病人在72小时后出院。随访期间血清肌酐未见明显变化。最终病理显示为透明细胞肾细胞癌,pT1a,大多数肿瘤ISUP 2级,但1例ISUP 3级。手术切缘阴性。结论:TP联合RP是治疗多发性肾肿瘤的可行选择。它是安全有效的,允许使用体内低温可以改善术后肾功能。在开始这个手术之前,需要持续的经验。
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Multiple renal tumorectomy in a Von Hipple Lindau patient. Combined retro/transperitoneal approach with intracorporeal hypotermia
ABSTRACT Objective & Introduction: To show the feasibility of a combined transperitoneal (TP) and retroperitoneal (RP) laparoscopic approach in a Von Hipple-Lindau (VHL) patient with multiple kidney tumors. VHL is an autosomal dominant inherited syndrome characterized by a high incidence of benign and malignant tumors and cysts in many organs. Renal cell carcinoma is one of the most common and a leading cause of mortality (1). Surgical approach is usually complex because of its multiplicity and the need of maximum kidney function preservation due to the risk of future recurrences (2, 3). Intracorporeal renal hypothermia may be useful in these cases to prevent permanent renal function loss (4). Materials and Methods: A 40 years old male was being monitored for multiple bilateral renal masses. Family history included a VHL syndrome affecting his mother and sister. Past medical history included a VHL syndrome with multiple cerebellar and medular hemangioblastomas, a pancreatic cystoadenoma and bilateral kidney tumors which had significantly grown up during follow-up. The patient was scheduled for laparoscopic multiple partial nephrectomy. A combined TP and RP approach with intracorporeal hypothermia was chosen. Results: A total of six right kidney tumors were removed. Operative time was 240 min. Cold ischemia time was 50 min. Average kidney temperature was 23.7°C. Blood losses were negligible. The patient was discharged after 72 hours. No major changes in serum creatinine were found during the follow-up. Final pathology revealed a clear cell renal cell carcinoma, pT1a, ISUP grade 2 in most of the tumors but one ISUP grade 3. Surgical margins were negative. Conclusions: Combined TP and RP is a feasible alternative for the treatment of multiple renal tumors. It's safe and effective, allowing the use of intracorporeal hypothermia which may improve postoperative renal function. Consistent experience is needed before embarking on this surgery.
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