Preoperative phase
The panel recommends expedited initiation of treatment for the management of WT(Strong recommendation, Quality of evidence: Very Low).
Panel deliberation. WT is a rapidly growing malignancy with a doubling time of less than 2 weeks. Therefore, any delay in diagnosis or initiation of therapy (Supplemental Table S3) should be avoided. To facilitate early identification of patients with WT, community awareness should be raised regarding the signs and symptoms of WT, favorable outcomes with timely treatment, and the urgency to seek health care. Strengthening referral networks and prioritizing chemotherapy and surgery for childhood cancer are essential to facilitate access to timely care.
The panel suggests surgery at a high-volume setting for patients with WT undergoing resection. (Weak recommendation, Quality of evidence: Very Low; Table 1).
Panel deliberation. Cancer units may have the capacity to offer common diagnostic and treatment services; however, pediatric oncologic surgery requires multidisciplinary capacity available at national cancer referral centers.11-15 When scaling-up surgical oncology care (Supplemental Table S2), it is fundamental to balance the competing priorities of quality and access. National cancer centers should be accessible and well connected with a network of primary health and cancer units to deliver affordable, equitable, and high-quality care. The cost effectiveness of treating cancer in centers with higher capabilities was shown in the third edition of Disease Control Priorities.16
The panel recommends abdominal and pelvic cross-sectional imaging for preoperative planning in patients with suspected WT (Strong recommendation, Quality of evidence: Very Low).
Panel deliberation. Cross-sectional imaging provides more in-depth knowledge of tumor anatomy, including focality and extent, which is important for preoperative surgical planning.17-22 Surgeons should develop skills to adequately interpret cross-sectional imaging delineating tumor extent and laterality for surgical planning. Although there is a paucity of evidence to compare outcomes of ultrasound-guided management versus cross-sectional imaging–guided management, the latter approach provides more comprehensive images of the tumor and its critical anatomy for the operating surgeon. When cross-sectional imaging is not readily available, abdominal ultrasound imaging may be used to guide therapy and provide valuable details about tumor origin, extent, and laterality.
The panel suggests chest computerized tomography (CT)-guided staging for the management of WT. (Weak recommendation, Quality of evidence: Very Low; Table 2)
Panel deliberation. Chest CT is significantly more sensitive than chest x-ray; management guided by chest CT is associated with improved event-free survival but not overall survival.23-27 When CT is not available, chest x-ray may guide therapy without compromising overall survival.
The panel recommends against biopsy for patients with typical clinical features of WT. (Strong recommendation, Quality of evidence: Very Low)
Panel deliberation. Children who present with typical clinical features of WT (Supplemental Table S3), including age (>6 months and <7 years), symptoms, laboratory test results, and imaging features, should receive neoadjuvant chemotherapy without tissue diagnosis. In the context of limited diagnostic capacity, routinely performing biopsies in patients with typical WT presentation may delay therapy and increase complications. Open biopsy can upstage WT, thereby compromising local control.28,29
The panel recommends biopsy (or upfront surgical resection when safe) for patients with renal tumor with an atypical clinical feature.(Strong recommendation, Quality of evidence: Very Low)
Panel deliberation. Other tumors that are not of renal origin, such as neuroblastoma and Burkitt lymphoma, should be excluded by clinical examination, laboratory investigations, and imaging. Patients who present with primary renal tumors but with clinical features that are atypical of WT should have tissue confirmation to plan therapy appropriately. Atypical presentations of WT include age older than 7 years or younger than 6 months, absence of imaging features that are typical of WT, infants with pulmonary metastases, extra-pulmonary/hepatic metastases, lactate dehydrogenase levels more than four times the normal limits, and hypercalcemia (Supplemental Table S3). In these scenarios, pre-therapy biopsy is indicated.30 Image-guided core needle biopsy, if available, is the approach of choice; open biopsy is associated with the risk of tumor spillage and should be avoided. Alternatively, if the tumor is deemed resectable, then upfront resection may be considered to provide tissue diagnosis and plan appropriate further treatment.