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Overall Survival and Renal Function of Patients with Bilateral Wilms Tumor Undergoing Surgery at a Single Institution
Andrew M Davidoff1, Rodrigo B. Interiano*1, Lynn Wynn*1, Noel Delos Santos*1, Jeffrey S. Dome*2, Rachel C. Brennan*1, M. Elizabeth McCarville*1, Matthew J. Krasin*1, Kathleen Kieran*3, Mark A. Williams*1
1St. Jude Children's Research Hospital, Memphis, TN;2Children's National Medical Center, Washington, DC;3University of Iowa Children's Hospital, Iowa City, IA

OBJECTIVE(S):
Approximately 5% of children with Wilms tumor present with bilateral disease, resulting in about 25 cases/year in the United States. The treatment challenge is to achieve a high cure rate while maintaining long-term renal function. We retrospectively reviewed our institutional experience with nephron-sparing surgery (NSS) in patients with bilateral Wilms tumor (BWT) between 2001-2014.
METHODS:
Imaging studies, surgical approach and pathology reports were reviewed. Outcomes evaluated included surgical complications, tumor recurrence, patient survival and renal function, as assessed by estimated glomerular filtration rate or radionuclide scans.
RESULTS:
Forty-two patients with BWT were identified; 39 (92.9%) patients underwent bilateral NSS and 3 underwent unilateral nephrectomy with contralateral NSS. One additional patient with a solitary kidney underwent NSS on that kidney. Acute post-operative complications included prolonged urine leak (10), hospital-acquired infection (6), intussusception (1) and transient renal insufficiency (1). Five patients required early (within four months) redo NSS for residual tumor. Overall survival was 88.4% (mean follow-up, 3.8 years). Of the 5 patients who died, 4 had disease of anaplastic histology (2 had focal anaplasia at initial resection, 2 had diffuse anaplasia at recurrence). Long-term, seven patients had local tumor recurrence (managed with redo NSS in 6 and completion nephrectomy in 1) and four had an episode of intestinal obstruction. All patients had GFR>60mL/min/1.73m2 at last follow-up.
CONCLUSIONS:
In patients with bilateral Wilms tumor, bilateral nephron-sparing surgery is safe and almost always feasible, thereby saving maximal renal parenchyma. With this approach, survival was excellent, as was maintenance of long-term renal function.


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