Yao Wei, Weng Shuting, Li Kai, Shen Jian, Dong Rui, Dong Kuiran
Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai, China.
Department of Pediatric Urology, Children's Hospital of Fudan University, Shanghai, China.
Transl Cancer Res. 2024 Feb 29;13(2):879-887. doi: 10.21037/tcr-23-1428. Epub 2024 Feb 27.
Nephron sparing surgery (NSS) had become the main surgical treatment for bilateral nephroblastoma. But it remained a challenge for surgeons to balance the dilemma between complete tumor resection to reduce tumor recurrence and renal parenchyma preservation to reduce end-stage renal disease (ESRD). In this study, we summarized our clinical experience for bilateral Wilms tumors managed in our center and evaluated the influence of different surgical approaches on prognosis.
The clinical data of patients with bilateral Wilms tumor in our hospital from January 2010 to December 2020 were retrospectively analyzed, and the clinical symptoms, surgical approaches and prognosis of the disease were summarized.
We reviewed medical records of 16 patients, including 13 (81.3%) girls and 3 (18.7%) boys. The mean age of the patients was 17.88±11.65 months (range, 6-42 months). Five patients presented with hypertension and two presented with hemihypertrophy. Fifteen cases had synchronous tumors, while only one patient had metachronous bilateral lesions. Thirteen patients received neoadjuvant chemotherapy and only 8 kidneys (30.8%) responded to chemotherapy. Two patients gave up surgery, and the other fourteen patients underwent radical resection, of which 2 patients only underwent unilateral radical nephrectomy (RN); 7 and 5 patients underwent single-stage and two-stage operation for bilateral lesions, respectively. In all surgical patients, RN was performed on 5 kidneys, and NSS was performed on 21 kidneys. The positive margins after NSS were found in 6 kidneys (35.3%). After a median follow-up period of 26.3 months, local tumor recurrence and renal insufficiency occurred in two and one patients. The 5-year overall and event-free survival rates were 78.1% and 58.6%, respectively. In univariable analysis, the survival rate in the initial chemotherapy group (92.3%) was significantly higher than that in the initial surgery group (33.3%) (P=0.048), whereas positive margin and staged operation (P>0.05) appeared not significantly associated with overall survival.
The proportion of tumor reduction after preoperative chemotherapy is relatively low for bilateral Wilms tumor, but preoperative chemotherapy could improve overall survival. NSS is recommended for bilateral Wilms tumor, and the customized procedure can be selected according to the location and anatomical features of tumor.
保留肾单位手术（NSS）已成为双侧肾母细胞瘤的主要手术治疗方式。但对于外科医生而言，在实现完整肿瘤切除以降低肿瘤复发与保留肾实质以减少终末期肾病（ESRD）之间寻求平衡仍具挑战性。在本研究中，我们总结了本中心治疗双侧Wilms肿瘤的临床经验，并评估了不同手术方式对预后的影响。
回顾性分析我院2010年1月至2020年12月期间双侧Wilms肿瘤患者的临床资料，总结该疾病的临床症状、手术方式及预后情况。
我们查阅了16例患者的病历，其中女孩13例（81.3%），男孩3例（18.7%）。患者的平均年龄为17.88±11.65个月（范围6 - 42个月）。5例患者出现高血压，2例出现半身肥大。15例为同步肿瘤，仅1例患者为异时性双侧病变。13例患者接受了新辅助化疗，仅8个肾脏（30.8%）对化疗有反应。2例患者放弃手术，其他14例患者接受了根治性切除，其中2例仅接受了单侧根治性肾切除术（RN）；7例和5例患者分别对双侧病变进行了一期和二期手术。在所有接受手术的患者中，5个肾脏进行了RN，21个肾脏进行了NSS。NSS术后切缘阳性在6个肾脏中发现（35.3%）。中位随访期26.3个月后，2例患者出现局部肿瘤复发，1例患者出现肾功能不全。5年总生存率和无事件生存率分别为78.1%和58.6%。单因素分析中，初始化疗组的生存率（92.3%）显著高于初始手术组（33.3%）（P = 0.048），而切缘阳性和分期手术（P>0.05）似乎与总生存率无显著相关性。
双侧Wilms肿瘤术前化疗后肿瘤缩小比例相对较低，但术前化疗可提高总生存率。双侧Wilms肿瘤推荐行NSS，可根据肿瘤位置及解剖特点选择个体化手术方式。
