Halim Amal, Elashry Rasha, Soliman Othman, Sarhan Osama
Department of Clinical Oncology and Nuclear Medicine, Faculty of Medicine, Mansoura University, Egypt.
J Oncol Pharm Pract. 2012 Mar;18(1):115-21. doi: 10.1177/1078155210396575. Epub 2012 Jan 10.
Bilateral Wilms' tumor (WT) is a challenge. Aggressive surgical resection is needed to prevent recurrence. We revised the clinico-epidemiological criteria of bilateral WT patients in our locality and relation to outcome.
462 WT patients were registered in three medical centers at Mansoura, Egypt. Twenty five patients had bilateral WT whose medical records were revised for all clinico-epidemiologic data plus treatment details, toxicity, and outcome.
The mean age was 34.5 months; 64% of cases were female. Abdominal mass was the commonest presentation (72%). Congenital anomalies were reported in two cases (one case showed hemihypertrophy and the other showed aniridia). About 60% had favorable pathology. Nineteen cases had synchronous bilateral WT (76%) and the remaining (six cases) had metachronous tumors. For the synchronous cases, the response rate to preoperative chemotherapy was 79% and nephron sparing surgery for the least involved kidney was possible in all. Survival rate was 74%. Metachronous tumor management included nephrectomy followed by chemotherapy for the initially diagnosed tumors. However, nephron sparing surgery of the contralateral tumors following preoperative chemotherapy was possible in two cases and the survival rate was 33%. No renal failure or any therapy-related complications were reported.
Bilateral WT is predominantly synchronous with favorable histology, with female predilection and possibly congenital anomalies. Preoperative chemotherapy followed by nephron sparing surgery has a favorable outcome with preserved renal function especially in patients with synchronous WT. Response to preoperative chemotherapy had a statistically significant prognostic impact.
双侧肾母细胞瘤(WT)是一项挑战。需要积极的手术切除以防止复发。我们修订了本地双侧WT患者的临床流行病学标准及其与预后的关系。
462例WT患者在埃及曼苏拉的三个医疗中心登记。25例为双侧WT,其病历针对所有临床流行病学数据以及治疗细节、毒性和预后进行了修订。
平均年龄为34.5个月;64%的病例为女性。腹部肿块是最常见的表现(72%)。报告了2例先天性异常(1例表现为半身肥大,另1例表现为无虹膜)。约60%具有良好的病理特征。19例为同时性双侧WT(76%),其余6例为异时性肿瘤。对于同时性病例,术前化疗的缓解率为79%,所有病例均可行对受累最轻的肾脏进行保留肾单位手术。生存率为74%。异时性肿瘤的治疗包括对最初诊断的肿瘤进行肾切除术,然后进行化疗。然而,2例患者在术前化疗后可行对侧肿瘤的保留肾单位手术,生存率为33%。未报告肾衰竭或任何与治疗相关的并发症。
双侧WT主要为同时性,组织学良好,女性多见,可能伴有先天性异常。术前化疗后行保留肾单位手术预后良好,肾功能得以保留,尤其是同时性WT患者。术前化疗的反应具有统计学上显著的预后影响。