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.