Wilms tumor: recent advances in clinical care and biology

doi:10.1053/j.sempedsurg.2011.10.002

Seminars in Pediatric Surgery

Volume 21, Issue 1, February 2012, Pages 15-20

https://doi.org/10.1053/j.sempedsurg.2011.10.002 Get rights and content

Wilms tumor is the most common renal malignancy that occurs in childhood. The remarkable evolution of treatment for this malignancy has served as a paradigm for multimodal cancer treatment. A rich database has accrued over time from well-controlled clinical trials. Translational research has directly affected patient care by allowing risk-based therapy. This article will summarize advances in our knowledge of the biology of Wilms tumor and describe the impact on clinical treatment of Wilms tumor.

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Cited by (34)

A Role for Genitourinary Reconstruction in Locally Advanced Bilateral Wilm's Tumor
2023, Urology
Wilm’s tumor, an embryonal tumor that develops from remnants of the immature kidney, is the most common primary malignant renal tumor of childhood and accounts for approximately 6%-7% of all childhood cancers. Here, we present a case of a 10-year-old female with stage IV Wilm’s tumor involving the bilateral kidneys. She ultimately required complex genitourinary reconstruction via a transureteroureterostomy following extensive oncologic resection. This case highlights the critical role of Urologists at an early stage in cases requiring complex genitourinary reconstruction.
Nephrectomy for Pediatric Renal Tumors: A Modified Surgical Approach to Minimize Post-operative Intestinal Obstruction
2023, Journal of Pediatric Surgery
The authors present a modified surgical technique during tumor nephrectomy in children with a conservative approach towards small bowel manipulation and cutting of the peritoneal reflections. We aimed to evaluate this modified surgical approach regarding the incidence of post-operative small bowel obstruction (SBO), and its technical utility.
The study includes all children with unilateral renal tumors who underwent radical nephrectomy and lymph nodes sampling at our tertiary center from 2010 to 2022. The modified technique was performed via the usual transverse incision. We cut the peritoneal reflections short of the cecum or short of the sigmoid colon. The colon is reflected over SB packing it, proceeding to nephrectomy and lymph nodes sampling. Data included demographics, clinical characteristics, treatment strategy, operative details, post-operative SBO, and overall outcomes.
The study included 890 patients with a median age of 3.2 years. The median tumor largest diameter was 13 cm (range: 9–18 cm). The modified surgical technique was adopted in 287 patients (32.3 %). Forty-three patients (43/890, 4.8 %) had post-operative SBO. Out of them, only 4 cases were operated on using the modified surgical technique (p-value<0.001). There were no significant differences between both techniques regarding timing of surgery, tumor rupture, lymph nodes sampling, and tumor size (p-value = 0.775, 0.328, 0.216, and 0.563, respectively).
The modified surgical approach is significantly correlated with lower incidence of post-operative SBO with no increased risk of tumor rupture or incomplete lymph nodes sampling. The timing of surgery or tumor characteristics had no significant impact on the technical utility of the modified surgical approach.
Level IV.
Bilateral Wilms’ tumour: An international comparison of treatments and outcomes
2021, Journal of Pediatric Surgery
Citation Excerpt :
Wilms’ tumour represents the most common renal malignancy of childhood, accounting for 90% of paediatric renal cancers, and around 7% of all childhood malignancy [1].
Introduction:
Wilms’ tumour is the most common childhood renal malignancy, with 5–10% of cases presenting bilaterally ¹. However, there is currently no consensus between centres on optimal management of bilateral Wilms’ tumours. This is an international multi-centre case series comparing management and outcomes of bilateral Wilms’ tumours between low-income centres (LIC) and high-income centres (HIC).
Methods:
Patients with bilateral Wilms' tumour were identified from four tertiary referral centres internationally. Data were collected on baseline characteristics, disease status, treatment used and clinical outcomes. Results were compared between individual centres as well as between groups of low-income centres (LIC) and high-income centres (HIC).
Results:
Data were collected for forty patients. Most patients received preoperative chemotherapy (n = 38, 95%). The most common surgical procedures were bilateral nephron-sparing surgery (n = 10, 25%) and nephrectomy with partial nephrectomy (n = 20, 50%). Ten-year survival after treatment was as follows: LIC's n = 13 (65%); HIC's n = 20 (100%) (p = 0.01).
Discussion:
Ten-year survival was significantly higher in HIC's. Our results show this may be caused by patient factors such as later presentation with more advanced disease in low-income centres. This comparative case series is the first to report on a large number of cases from multiple international centres, and to compare key outcomes.
Global surgery
2020, Journal of Pediatric Surgery
Citation Excerpt :
A large multicentre cohort study on bilateral Wilms' tumour (BWT) was presented comparing management of BWT between patients from HIC and LMIC. These tumours are the most common renal malignancy of childhood in both HIC and LMIC and BWT make up 5–10% of these [14]. Participating countries included UK, New Zealand, South Africa and Nigeria.
This article outlines the global surgery activities held at the 66th Annual International Meeting of the British Association of Paediatric Surgeons (BAPS) in 2019. For the first time BAPS introduced a plenary session on global surgery. Topics that were covered include management of birth defects in Low-Middle Income Countries (LMIC), mostly gastroschisis, and community-based involvement in children's surgery, knowing that 2.5 billion children worldwide do not have access to surgical care. The presentation on financial burden for families with children needing surgical care highlighted the route to poverty in the absence of governmental support. The Hugh Greenwood lecture covered the involvement of a global organisation, namely Global Initiative for Children's Surgery (GICS) advocating and working towards children's surgery, as this was ignored at the Lancet Commission held in 2015.
Level V.
General Abdominal and Urologic Surgery
2019, A Practice of Anesthesia for Infants and Children
General Abdominal and Urologic Surgery
2018, A Practice of Anesthesia for Infants and Children

View all citing articles on Scopus

View full text

Wilms tumor: recent advances in clinical care and biology

Ann Oncol

J Pediatr Surg

J Pediatr Surg

J Pediatr Surg

J Urol

Wilms' tumours: About tumour suppressor genes, an oncogene and a chameleon gene

Nat Rev Cancer

Position paper: Imaging methods for primary renal tumors of childhood: Costs versus benefits

Med Pediatr Oncol

Surgery-related factors and local recurrence of Wilms tumor in National Wilms tumor study 4

Ann Surg

Nephroblastoma: Does the decrease in tumor volume under preoperative chemotherapy predict the lymph node status at surgery?

Pediatr Blood Cancer

Reduction of postoperative chemotherapy in children with stage I intermediate-risk and anaplastic Wilms' tumour (SIOP 93-01 trial): A randomised controlled trial

Lancet

Loss of heterozygosity for chromosomes 1p and 16q is an adverse prognostic factor in favorable-histology Wilms tumor: A report from the National Wilms Tumor Study Group

J Clin Oncol

Treatment with nephrectomy only for small stage I/favorale histology Wilms' tumor: A report from National Wilms Tumor Study Group

J Clin Oncol

Long-term outcomes for infants with key low risk Wilms tumor treated with surgery alone in National Wilms tumor study-5

Ann Surg