Northeast Florida Medicine
Vol. 66, No. 2 2015
43
CME
study, whose materials will be analyzed for risk factors for
recurrence to modify treatment for certain risk groups
in future trials.
Standard Risk
The standard risk group is a heterogeneous population
consisting of stage I-II patients with LOH 1p/16q, and
stage III patients without LOH 1p/16q. Stage I-III pa-
tients in this category can be treated per AREN 0532,
in which patients receive surgery, followed by adjuvant
DD4A chemotherapy (actinomycin-D, vincristine, and
doxorubicin). Stage III patients also receive adjuvant
flank or abdominal radiation therapy. Some Stage IV
patients have also been classified as ‘Standard Risk’ if they
experienced a rapid complete response to preoperative
chemotherapy. If so, similar chemo- and radiotherapy
treatments are applied, but pulmonary radiation was
often omitted.
Higher Risk
The remaining stage III and IV patients with favorable
histology tumors fall into this category and are treated
per AREN 0533, which intensifies chemotherapy in
patients failing to respond adequately by incorporating
cyclophosphamide and etoposide.
Bilateral
Patients with bilateral Wilms tumor (BWT), or pa-
tients with unilateral Wilms tumor who are high risk
for developing BWT, can be enrolled in AREN 0534
which is currently active. The goal of this study is to
use preoperative chemotherapy (three-drug regimen for
BWT, two-drug regimen for unilateral) to allow tumor
shrinkage to occur and increase the success rate of partial
nephrectomy, in order to preserve renal function.
High Risk
These patients are being evaluated on AREN 0321,
which closed to accrual in November 2013, and includ-
ed all patients with anaplastic Wilms tumors, and other
malignant renal tumors (malignant rhabdoid tumor, clear
cell sarcoma kidney, and renal cell carcinoma). This study
is evaluating different chemotherapy regimens, and all
patients with Wilms tumor receive radiation to the flank
and sites of metastatic disease (if present).
Recurrent Disease
At present, there is no standard management strategy
for recurrent Wilms tumor. The most common strate-
gies employ multi-agent chemotherapy with ifosfamide,
carboplatin and etoposide, which has raised survival to
approximately 50 percent
12
. Much remains unanswered,
including the value of re-resection of primary and meta-
static disease
13
, and the value of high dose therapy with
autologous stem cell rescue.
14
Final Thoughts
Treatment of Wilms tumor remains one of pediatric
oncology’s great successes and is one of the best examples
of what is achievable by thoughtful, regimented, multi-
disciplinary oncologic care. With pediatric tumors being
(relatively) rare, pediatric oncology has long embraced
the notion of treating every child on protocol. Thus,
even with a tumor as uncommon as Wilms tumor, steady
progress continues to be made. Novel therapeutics such as
topotecan or anti-angiogenesis agents such as bevacizumab
are being evaluated, and will hopefully prove of benefit
in the higher risk and recurrent groups for whom cure
remains elusive.
Treatment of Wilms is a true test of an oncologic
program, as it hinges on cooperation of all aspects of
the program— radiology, surgery, pathology, medical
oncology, and radiation oncology. Coordination and
communication with one another is imperative to prop-
erly stage and risk stratify the patient. With cure rates in
Wilms at record highs, expectations are equally high.
v
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