Laparoscopic nephron-sparing resection of synchronous Wilms tumors in a case of hyperplastic perilobar nephroblastomatosis

Abstract

Diffuse hyperplasticperilobarnephroblastomatosis (DHPLN) is a rare precursor lesion of Wilms tumor (WT). Because of the increased risk to develop WT in either kidney, current management algorithms of DHPLN meritnephron-sparing strategies, beginning with chemotherapy and close radiographic monitoring into late childhood. After resolution of DHPLN, subsequent detection of a renal nodule mandates resection to exclude WT. Here, we report the case of a 4 year-old girl who developed two synchronous nodules in the right kidney more than two years after completion of therapy for DHPLN. Because of the early detection and peripheral location of these two nodules, laparoscopic nephron-sparing resection of each was performed using ultrasonic dissection. Both nodules were determined on pathology to be favorable histology WT with negative surgical margins. The child was placed onvincristine and actinomycin-D therapy for 18 weeks. KeywordsWilms tumor; nephroblastomatosis; laparoscopy; nephron-sparing surgery Diffuse hyperplasticperilobarnephroblastomatosis (DHPLN) is a rare precursor lesion of Wilms tumor (WT)and may be considered an intermediate, pre-neoplastic stage in the sequence of Wilms tumorigenesis(1-5).Because the vast majority of DHPLN cases occur with bilateral renal involvement, current treatment algorithms emphasize nephron-sparing strategies(4).At time of diagnosis, DHPLN patients are recommended to receive 18 weeks of vincristine and actinomycin-D and may continue on extended therapy until all lesions resolve(5,6). Refractory, recurrent or new expanding nodules prompt tissue diagnosis with nephron-sparing resection to exclude WT initiation. Notably, in the seminal report outlining the natural history of DHPLN and the risk to develop WT, 24 of 52 (46.2%) patients who Address correspondence to: Harold "Bo" N. Lovvorn, III, MD, FACS, FAAP, Assistant Professor of Pediatric Surgery, Vanderbilt University Children's Hospital, Doctor's Office Tower, Suite 7102, 2200 Children's Way, Nashville, TN 37232-9780, Office Phone: 615-936-1050, Facsimile: 615-936-1046.lovvorn@vanderbilt.edu. Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. were followed for a minimum of five years developed WT, and 11 of these children developed two or more WT(4). Because of this risk to develop multiple WT in either kidney, conservative surgical strategies need to be followed to minimize late renal insufficiency, dialysis dependence, and need for renal transplantation(7). NIH Public AccessNephron-sparing strategies have been described for children presenting with bilateral WT(7-9).However, no des...

Current surgical standards of care in Wilms tumor

“…Minimally invasive NSS for WTs has also been published, although at least one patient had diffuse peritoneal metastases following laparoscopic NSS [28][29][30].…”

Robotic‐assisted laparoscopic management of renal tumors in children: Preliminary results

“…NSS for unilateral WT has long been widely accepted in syndromic patients such as Denys–Drash, WAGR, and other WT1‐related diseases and more recently applied to any unilateral WT that fulfilled the criteria of the Umbrella protocol . In our experience, nephron‐sparing surgery is usually performed by a transperitoneal open approach.…”

“…NSS for unilateral WT has long been widely accepted in syndromic patients such as Denys-Drash, WAGR, and other WT1-related diseases [28][29][30] and more recently applied to any unilateral WT that fulfilled the criteria of the Umbrella protocol. 12 In our experience, The benefits of this technique compared with classic laparoscopy may come to light after many years, if they do at all; but the medico-economic study linked to our robotic program, which is currently running, will certainly help in evaluating cost-efficiency.…”

Nephron-sparing surgery for bilateral Wilms tumor

“…Laparoscopic partial nephrectomy for WT has been reported, 22–23 although there is at least one report of extensive peritoneal involvement with tumor following minimally invasive NSS. 24 At present, an open surgical approach remains the standard of care for NSS in patients with BWT.…”