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Bilateral Wilms' tumors: single-center experience with 22 cases and literature review.

机译:双侧Wilms肿瘤:单中心经验22例并文献复习。

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OBJECTIVES: Bilateral Wilms' tumors represent a therapeutic challenge. The primary aim of management is eradication of the neoplasm and preservation of renal function. We present our experience in the management of such cases in a single-center experience. METHODS: This was a retrospective study of 22 patients with histologically proven bilateral nephroblastoma who were treated from 1993 to 2008 at our center. Of the 22 patients, 12 were girls and 10 were boys, with a median age of 3 years (range 1-9); 19 had a synchronous presentation and 3 a metachronous presentation. Of the 22 patients, 6 underwent initial surgical resection followed by chemotherapy and 16 underwent initial biopsy and preoperative chemotherapy. The final oncologic and renal outcomes were assessed. RESULTS: The median follow-up period was 3 years (range 1-11). Of the 22 patients, 8 died, for an overall survival rate of 63.5%. The survival for the initial chemotherapy and initial surgery groups was essentially similar. Of all the variables studied, unfavorable histologic findings had a significant negative effect on survival. Of the 5 patients with unfavorable histologic findings, 4 died during the follow-up period. The median volume of preserved renal parenchyma was 40%. All patients had good renal function during follow-up, except for 1 patient who had undergone bilateral nephrectomy. CONCLUSIONS: Bilateral Wilms' tumors impose 2 conflicting issues: elimination of the pathology and preservation of the renal function. Currently, treatment regimens involving initial chemotherapy followed by conservative surgery can achieve these goals in an important proportion of patients.
机译:目的:双侧Wilms肿瘤代表治疗挑战。管理的主要目的是根除肿瘤和保持肾功能。我们在单中心的经验中介绍我们在处理此类案件方面的经验。方法:这是一项回顾性研究,对1993年至2008年在我们中心接受治疗的22例经组织学证实为双侧肾母细胞瘤的患者进行了回顾性研究。在22名患者中,女孩为12名,男孩为10名,中位年龄为3岁(范围1-9); 19个具有同步演示文稿,3个具有同步演示文稿。在22例患者中,有6例接受了首次手术切除,然后进行了化疗,还有16例接受了初步活检和术前化疗。评估了最终的肿瘤和肾脏结局。结果:中位随访期为3年(范围1-11)。在22例患者中,有8例死亡,总生存率为63.5%。初始化疗组和初始手术组的生存率基本相似。在所有研究的变量中,不利的组织学检查结果对存活率有明显的负面影响。在5例组织学检查结果不良的患者中,有4例在随访期间死亡。保留肾实质的中位体积为40%。除1例行双侧肾切除术的患者外,所有患者在随访期间均具有良好的肾功能。结论:双侧Wilms肿瘤具有两个相互矛盾的问题:消除病理学和保留肾功能。当前,涉及初始化疗然后进行保守手术的治疗方案可以在很大比例的患者中实现这些目标。

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