Evidence-Based Surgical Guidelines for Treating Children with Wilms
Tumor in Low-Resource Settings
Abstract
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 non-anaplastic histology stage
IV WT with pulmonary metastasis. Conclusion: We provide evidence-based
recommendations for the surgical management of WT, considering the
benefits/risks associated with limited-resource settings.