Department of Pediatric Surgery, Aga Khan University Hospital Karachi, Stadium Road, Karachi, 74800, Sindh, Pakistan.
St Jude Children's Research Hospital Memphis, Karachi, 74800, Sindh, Pakistan.
BMC Pediatr. 2024 Oct 10;24(1):645. doi: 10.1186/s12887-024-05122-1.
The aim of this study was to assess management and determine outcomes of renal tumors with inferior vena cava (IVC) and intracardiac (IC) extension in a tertiary care center in Pakistan.
A retrospective chart review was conducted at the Aga Khan University Hospital, Karachi, Pakistan. All patients from 1 to 18 years of age with renal tumors with intravascular extensions, surgically managed from January 1988 till June 2016, were included. Data was extracted by reviewing medical records, and the tumor details, treatment and outcomes were analyzed.
A total of 18 patients out of the total 61 patients with renal tumors, presented with IVC and/or IC extension, with the majority involving the right kidney. Mean age was 5.9 (SD:4.9) and a female preponderance (56%) was seen. Wilms tumor (77%) was the most common tumor type, with the level of tumor extension into IVC predominantly being below the diaphragm (55.5%). Fourteen patients received preoperative chemotherapy, with tumor regression, seen in 10. Most patients underwent thrombectomy through the renal vein (56%). Regarding outcomes, frequency of mortality and morbidity was 1 and 2, respectively, with 7 patients having no recurrent 5 years post-surgery.
A greater incidence (29.5%) of IVC and or IC Tumor extension was found compared to existing literature, which could likely be due to a higher referral rate to the center. Moreover, this is a single-center study and so a multi-center study is crucial to form an assessment of surgical management in resource-limited settings. Our study is the first from Pakistan on this particular renal tumor presentation. Considering the varying case presentations and surgical techniques used, further studies are needed to standardize surgical management and optimize patient outcomes.
本研究旨在评估在巴基斯坦一家三级护理中心治疗肾肿瘤伴下腔静脉（IVC）和心内（IC）延伸的管理方法并确定其结局。
在巴基斯坦卡拉奇的 Aga Khan 大学医院进行了回顾性病历审查。纳入 1988 年 1 月至 2016 年 6 月期间在该中心接受手术治疗的所有年龄在 1 至 18 岁之间伴血管内延伸的肾肿瘤患者。通过查看病历提取数据，分析肿瘤详细信息、治疗和结局。
在 61 例肾肿瘤患者中，共有 18 例患者存在 IVC 和/或 IC 延伸，其中大多数涉及右肾。平均年龄为 5.9 岁（标准差：4.9），女性占多数（56%）。最常见的肿瘤类型是 Wilms 瘤（77%），肿瘤向 IVC 延伸的水平主要位于膈肌以下（55.5%）。14 例患者接受了术前化疗，其中 10 例肿瘤消退。大多数患者通过肾静脉进行血栓切除术（56%）。在结局方面，死亡率和发病率分别为 1%和 2%，7 例患者术后 5 年无复发。
与现有文献相比，我们发现 IVC 和或 IC 肿瘤延伸的发生率更高（29.5%），这可能是由于向该中心的转诊率更高。此外，这是一项单中心研究，因此多中心研究对于评估资源有限环境下的手术管理至关重要。我们的研究是巴基斯坦首例关于该特定肾肿瘤表现的研究。鉴于不同的病例表现和手术技术的使用，需要进一步研究来规范手术管理并优化患者结局。
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