Evidence‐based surgical guidelines for treating children with Wilms tumor in low‐resource settings

Abstract

Background Survival of Wilms tumor (WT) is > 90% in high‐resource settings but < 30% in low‐resource settings. Adapting a standardized surgical approach to WT is challenging in low‐resource settings, but a local control strategy is crucial to improving outcomes. Objective Provide resource‐sensitive recommendations for the surgical management of WT. Methods We performed a systematic review of PubMed and EMBASE through July 7, 2020, and used the GRADE approach to assess evidence and recommendations. Recommendations Initiation of treatment should be expedited, and surgery should be done in a high‐volume setting. Cross‐sectional imaging should be done to optimize preoperative planning. For patients with typical clinical features of WT, biopsy should not be done before chemotherapy, and neoadjuvant chemotherapy should precede surgical resection. Also, resection should include a large transperitoneal laparotomy, adequate lymph node sampling, and documentation of staging findings. For WT with tumor thrombus in the inferior vena cava, neoadjuvant chemotherapy should be given before en bloc resection of the tumor and thrombus and evaluation for viable tumor thrombus. For those with bilateral WT, neoadjuvant chemotherapy should be given for 6–12 weeks. Neither routine use of complex hilar control techniques during nephron‐sparing surgery nor nephron‐sparing resection for unilateral WT with a normal contralateral kidney is recommended. When indicated, postoperative radiotherapy should be administered within 14 days of surgery. Post‐chemotherapy pulmonary oligometastasis should be resected when feasible, if local protocols allow omission of whole‐lung irradiation in patients with nonanaplastic histology stage IV WT with pulmonary metastasis without evidence of extrapulmonary metastasis. Conclusion We provide evidence‐based recommendations for the surgical management of WT, considering the benefits/risks associated with limited‐resource settings.

The role of Pediatric Surgery in childhood cancer

“…The expertise and ongoing training of pediatric surgeons are also critical in managing surgical complications in pediatric oncology patients, requiring technical skills and a deep understanding of treatment guidelines and care specific to this population. Additionally, adapting standardized surgical approaches in resource-limited settings poses challenges, highlighting the importance of specialization and training in devising local control strategies to improve outcomes 13 .…”

“…These challenges are particularly acute in low- and middle-income countries (LMICs), where limited resources and access to training can impede the widespread adoption of robotic surgery. Additionally, the learning curve associated with these techniques necessitates ongoing education and skill development among surgical teams 13 , 15 , 19 .…”

“…Despite their critical role, access to pediatric surgical oncology training is limited globally, especially in low- and middle-income countries. This highlights the need for further research and education to develop a global workforce capable of providing high-quality pediatric cancer care 7 , 13 . The role of pediatric surgery in childhood cancer outcomes is the focus of this article, which aims to provide an overview of the state-of-the-art and the future directions of this field.…”

Wilms Tumor: Updates about Pathogenesis and New Possible Clinical Treatments of the Most Frequent Pediatric Urogenital Cancer: A Narrative Review

“…Radiotherapy is given to children who have the disease at a higher stage (stage III and in the presence of distant metastases, usually of the lungs, which do not regress readily with chemotherapy). In most cases, radical nephrectomy is practiced, i.e., the surgical removal of the affected kidney, associated with the resection of the regional and para-aortic lymph nodes ipsilateral to the neoplasm; in bilateral tumors or patients with specific syndromes predisposed to the onset of nephroblastoma, partial nephrectomy is to be preferred whenever feasible [39][40][41][42][43][44]; when possible, especially in the case of bilaterality, preference should be given to even partial preservation of the renal structure, unless clinical conditions permit and the balance with the renal function to be preserved is compatible with possible tumor recurrence [45]. An improvement in the clinical picture and renal parenchyma over historical outcomes emerges in children with the bilateral form (but not in diffuse anaplasia and inhomogeneous tumors) if the treatment approach includes standardized three-drug preoperative chemotherapy, surgical resection within 12 weeks of diagnosis and response and postoperative therapy based on the histologic picture [46][47][48][49][50][51]; still, in the experimental phase, one study showed that concomitant administration of WT1-immunotherapy and standard neoadjuvant therapy (used in breast cancer) was well tolerated and induced WT1-specific antibodies in patients receiving aromatase inhibitors in the neoadjuvant phase (precisely for patients with Wilms tumor); however, in patients receiving neoadjuvant chemotherapy or the trastuzumab-chemotherapy combination, the humoral response was impaired or attenuated, probably due to the co-administration of corticosteroids and/or the chemotherapeutics themselves [52].…”

Late health outcomes among survivors of Wilms tumor

“…In many of these lower-resource countries and areas, lack of access to chemotherapeutics is a major cause. Guidelines for these lower access areas frequently involve radiation therapy if available ( 9 ). This likely subsequently pushes those individuals into higher category risk for developing CHCs from their treatment.…”