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Management of Bilateral Wilms’ Tumor: Our Experience

机译:双边肿瘤的管理:我们的经验

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摘要

Management of bilateral Wilms’ tumor is particularly challenging, considering the chances of recurrence and long-term renal function for affected patients. Aggressive surgical resection to prevent recurrence must be balanced with the desire to preserve renal function. We evaluated our experience in the management of bilateral Wilms’ tumor stressing the challenges encountered in decision making and the role of nephron sparing surgery. We had four children presenting with bilateral Wilms’ tumor. All of them were appropriately staged and given standard chemotherapy as per NWTS-5 guidelines. Tumors were considered to have a ‘good’ response to chemotherapy if sufficient tumor shrinkage was observed so that renal hilum was seen free of tumor and vice versa. Nephron-sparing surgery was considered in all and was performed when feasible, followed by completion adjuvant chemotherapy. All patients were followed up with serial ultrasound scans (3–6 monthly) and CECT abdomen (yearly once). Blood urea and serum creatinine, hypertension, and proteinuria were assessed during follow-up visits. All four children received neo-adjuvant chemotherapy as per NWTS-5 guidelines. The first child had poor response to chemotherapy and was considered for left radical and right partial nephrectomy. However, patient attenders refused any surgical intervention and the child was taken home. The second child had a ‘good’ response on left side and was planned for left partial and right radical nephroureterectomy based on pre-operative imaging analysis. However, intra-operatively, bilateral partial nephrectomy with good margins was feasible. The third child though, showed ‘poor’ response to pre-op chemotherapy and warranted bilateral nephroureterectomy, right partial and left radical nephroureterectomy was feasible. However, in the fourth child, we were not able to perform nephron sparing surgery and left nephroureterectomy with right tumor biopsy was done. Following this, child was started on 2nd-line chemotherapy, now awaiting right partial nephrectomy. The second child is on follow up for 1.5 years, doing well. However, the third child expired 1.5 years following surgery due to recurrence (lung metastasis). Management of bilateral Wilm’s tumor is challenging and nephron-sparing surgery should be considered in all patients having bilateral Wilm’s tumor with favorable histology, even if pre-operative imaging studies suggest that the lesions are unresectable.
机译:考虑到患病患者复发和长期肾功能的机会,处理双侧Wilms肿瘤特别具有挑战性。积极进行手术切除以防止复发必须与保持肾功能的愿望相平衡。我们评估了在处理双侧Wilms肿瘤方面的经验,强调了决策过程中遇到的挑战以及肾单位保留肾手术的作用。我们有四个孩子患有双侧Wilms肿瘤。根据NWTS-5指南,对所有患者进行适当分期并给予标准化疗。如果观察到足够的肿瘤缩小,则认为肿瘤对化疗具有“良好”的反应,从而使肾门可见无肿瘤,反之亦然。总体上考虑了保留肾单位的手术,并在可行的情况下进行,然后完成辅助化疗。所有患者均接受连续超声扫描(每月3–6次)和CECT腹部(每年一次)随访。在随访期间评估了血尿素和血清肌酐,高血压和蛋白尿。根据NWTS-5指南,所有四名儿童均接受了新辅助化疗。第一个孩子对化学疗法的反应较差,因此考虑进行左根和右部分肾切除术。但是,病人护理人员拒绝任何手术干预,并将孩子带回家。第二个孩子的左侧反应良好,并根据术前影像学分析计划进行左侧部分和右侧根治性肾切除术。然而,术中双侧部分肾切除术具有良好的切缘是可行的。不过,第三个孩子对术前化疗表现出“差”的反应,需要进行双侧肾切除术,右部分和左根治性肾切除术是可行的。但是,在第四个孩子中,我们无法进行保留肾单位的手术,并进行了右肾活检的左肾切除术。此后,孩子开始接受二线化疗,现在正在等待右部分肾切除术。第二个孩子随访1.5年,状况良好。但是,由于复发(肺转移),第三个孩子在手术后1.5年就过期了。双侧Wilm's肿瘤的治疗具有挑战性,并且即使术前影像学检查表明病灶无法切除,也应考虑对所有组织学良好的双侧Wilm's肿瘤患者进行保留肾单位的手术。

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