JOURNAL OF SHANGHAI JIAOTONG UNIVERSITY (MEDICAL SCIENCE)

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Surgical and oncological outcome of 79 consecutive patients with unilateral favorable histology Wilms tumor treated in one single institution following an upfront tumor surgical strategy

XIE Chen-jie, GAO Yi-jin, YIN Min-zhi, CAI Jiao-yang, ZHANG An-an, TANG Yue-jia, GU Song, PAN Ci, TANG Jing-yan, XU Min   

  1. 1.Department of General Surgery/Oncological Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China;2.Department of Hematology/Oncology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China; 3.Department ofPathology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China
  • Received:2021-04-07 Published:2021-09-23
  • Contact: XU Min, E-mail: xumin@scmc. com. cn.
  • Supported by:
    None

Abstract: Objective·To improve the understanding of upfront surgical strategy in the treatment of Wilms tumor(WT) and standardize the upfront resection procedure and subsequent chemotherapy and/or radiotherapy approach, providing necessary data support for clinical study in childhood WT in China. Method·From January 1, 2010 to December 30, 2017, the clinical records of patients with unilateral favorable histology WT(FHWT) who were initially diagnosed at Shanghai Children’s Medical Center, Shanghai Jiao Tong University School of Medicine were retrospectively collected. All cases were diagnosed, staged and treated according to the Chinese Children Cancer Group(CCCG)-WT-2009 or CCCG-WT-2015 Protocol. Surgical outcome,event-free survival(EFS) and overall survival(OS) were analyzed using SPSS software, version 25.0. Follow-up was updated to March 31, 2020. Result·A total of 79 consecutive cases with unilateral FHWT were enrolled, including 41 males and 38 females, with a median age of 27.1 months. Systemic staging showed that there were 7 cases with stage Ⅰ, 39 cases with stage Ⅱ, 23 cases with stage Ⅲ and 10 cases with stage Ⅳ. Sixty-nine patients(87.3%) underwent nephrectomy and 10 patients(12.7%) underwent biopsy of renal tumor tissue(2 with core needle biopsy and 8 with open biopsy) in the primary operation. Eleven cases experienced disease progression/recurrence and 1 abandoned treatment and returned to original treatment plan after 8 weeks. Local radiation therapy was applied to 27 of 32 patients with indication. Only 3 of 10 patients with lung metastasis at diagnosis received whole lung radiation therapy. A median follow-up of 69 cases without incident was 69.8 months. The 5-year EFS and OS of all cases were 84.8% and 92.4%respectively. The 5-year EFS of children with stage Ⅰ, Ⅱ, Ⅲ and Ⅳ were 100.0%, 84.6%, 95.7% and 50.0%(P=0.010), respectively. And the 5-year OS of children with stage Ⅰ, Ⅱ, Ⅲ and Ⅳ were 100.0%, 94.9%, 95.7 and 70.0%(P=0.070), respectively. The 5-year EFS of children with stage Ⅲ/Ⅳreceiving initial nephrectomy was higher than that of those undergoing biopsy(91.3% vs 60.0%, P= 0.032). The 5-year EFS of children with initial biopsy, who underwent nephrectomy at week 6 was significantly higher than that of those who did not(100% vs 0, P=0.005). Conclusion·The upfront tumor surgical strategy of unilateral WT is safe, feasible and effective in the treatment of FHWT. Initial nephrectomy and clearance of all local diseases with lymph nodal sampling is the key to staging and successful treatment.

Key words: Wilms tumor, favorable histology, treatment, prognosis, child

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