Bilateral Renal Cancer in Children: A Difficult, Challenging and Changing Management Problem (original) (raw)
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Part II: Treatment of primary malignant non-Wilms' renal turnours in children
Lancet Oncol 8 842 848, 2007
Renal-cell carcinoma, clear-cell sarcoma, (congenital) mesoblastic nephroma, rhabdoid tumour, and renal medullary carcinoma form a heterogeneous group of childhood renal malignancies known as non-Wilms' tumours. Progress has been slow in improving the management of these tumours to decrease morbidity and increase survival. However, greater cooperation between national and international centres should engender specialisation, and an increased knowledge of the molecular biology of these tumours will inevitably lead to substantial progress over the next decade. This review is the second of two parts: the fi rst part provided an updated review of the clinical presentation, imaging, and pathology of non-Wilms' tumours and this second part provides an updated review of the treatment of these tumours.
Part II: Treatment of primary malignant non-Wilms' renal tumours in children
The Lancet Oncology, 2007
Renal-cell carcinoma, clear-cell sarcoma, (congenital) mesoblastic nephroma, rhabdoid tumour, and renal medullary carcinoma form a heterogeneous group of childhood renal malignancies known as non-Wilms' tumours. Progress has been slow in improving the management of these tumours to decrease morbidity and increase survival. However, greater cooperation between national and international centres should engender specialisation, and an increased knowledge of the molecular biology of these tumours will inevitably lead to substantial progress over the next decade. This review is the second of two parts: the fi rst part provided an updated review of the clinical presentation, imaging, and pathology of non-Wilms' tumours and this second part provides an updated review of the treatment of these tumours.
Renal transplant in a child with bilateral Wilms' tumor national premiere
Journal of medicine and life, 2008
Introduction: Bilateral Wilms' tumors with an unfavorable histology require a combined treatment (extensive surgery, polychimiotherapy, radiotherapy). Objective: Presentation of the first renal transplant performed in Romania in a child with bilateral Wilms' tumor, at 3 years and 4 months after the end of a multimodal treatment. Material and methods: Patient C. N., born on 30.04.1998, was diagnosed in 04.2001 with right parenchymal renal tumor, polycystic kidney, left cystic renal tumor. 25.04.2001-right radical nephrectomy and partial left upper pole nephrectomy; histopathology examination: triphasic bilateral nephroblastoma, reactive lymph nodes, negative resection edges in the left kidney. 30.04-19.11.2001-polychemotherapy according to the NWTS-5 stages 2-4 focal anaplasia and radiotherapy of the right kidney bed (29.06.2001). 02.2002-a nephrotic syndrome on the remnant kidney which requires its excision and peritoneal dialysis. Abdominal control CT was normal in 03.2005. 11.03.2005-renal transplant from living related donor. Results: Favorable post-transplant course with normal renal clearance values; at 2 months, normal urography control. Conclusions: The tumor pathology does not represent an absolute contraindication for renal transplantation. For the cases with extensive surgery, polychimiotherapy and radiotherapy correctly applied, a pre-transplant "tumorfree" period of at least 2 years is compulsory.
Journal of Pediatric Surgery, 1992
g The National Wilms' Tumor Study 3 (NWTS-3) recommended treatment of bilateral Wilms' tumor with initial biopsy followed by chemotherapy with subsequent operation to resect the remaining tumor. This study was performed to determine if this approach preserves renal mass and function when compared with initial surgical resection followed by chemotherapy. Over a PO-year period (1970 to IggO), 15 patients with synchronous bilateral Wilms' tumor were treated at the Childrens Hospital of Los Angeles. Eight patients in the surgical group underwent initial unilateral nephrectomy with contralateral biopsy, wedge resection, or partial nephrectomy and subsequent chemotherapy. The seven patients in the chemotherapy group underwent bilateral tumor biopsy, followed by chemotherapy and subsequent tumor resection. Patients were assigned to each group in a nonrandomized manner according to the preference of the attending oncologist and surgeon. Comparison of the two groups showed no significant differences in sex distribution, initial renal function, tumor histology, dose and field of radiotherapy, presence or absence of positive surgical margins, and local recurrence rates. Patients in the surgery group were slightly older than those in the chemotherapy group: 3.6 f 2.2 versus 2.3 f 2.2 years. The percent of renal mass involved by tumor for the surgery group was 52 f 12 versus 73 2 16 for the chemotherapy group (P = .03). The percent of renal mass preserved following all operations, local recurrence rates, incidence of metastases, and survival was nearly identical between the two groups. There were three cases of renal failure in the surgery group and one case of renal failure in the chemotherapy group. Two of the three patients in the surgery group with renal failure had sequential nephrectomies for their Wilms' tumor. In both cases an initial nephrectomy was performed on the side with the largest tumor. These data suggest that in the treatment of synchronous bilateral Wilms' tumor, the incidence of local recurrence, metastases, and survival are similar with either therapeutic approach. Despite a significantly greater percent of renal mass involved by tumor in the chemotherapy group, more cases of renal failure were observed in the group treated with initial operation. In order to prevent renal failure in these patients, initial unilateral nephrectomy should be avoided and long-term renal function should be monitored.
Journal of Pediatric Urology, 2020
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Journal of Clinical Medicine
Survival of unilateral Wilms tumors (WTs) is exceeding 90%, whereas bilateral WTs have an inferior outcome. We evaluated all Dutch patients with bilateral kidney tumors, treated in the first five years of national centralization and reviewed relevant literature. We identified 24 patients in our center (2015–2020), 23 patients had WT/nephroblastomatosis and one renal cell carcinoma. Patients were treated according to SIOP-RTSG protocols. Chemotherapy response was observed in 26/34 WTs. Nephroblastomatosis lesions were stable (n = 7) or showed response (n = 18). Nephron-sparing surgery was performed in 11/22 patients undergoing surgery (n = 2 kidneys positive margins). Local stage in 20 patients with ≥1 WT revealed stage I (n = 7), II (n = 4) and III (n = 9). Histology was intermediate risk in 15 patients and high risk in 5. Three patients developed a WT in a treated nephroblastomatosis lesion. Two of 24 patients died following toxicity and renal failure, i.e., respectively dialysis-r...
European Journal of Cancer, 2009
We prospectively evaluated tumour response and renal function in 12 newly-diagnosed children with high-risk Wilms tumour receiving ifosfamide, carboplatin, and etoposide (ICE) chemotherapy. Two cycles of ICE were followed by 5 weeks of vincristine, dactinomycin, and doxorubicin (Adriamycin) (VDA), and nephrectomy, radiotherapy, additional VDA, and a third ICE cycle. Carboplatin dosage was based on glomerular filtration rate (GFR) to achieve targeted systemic exposure (6 mg/ml × min). Mean GFR (measured by technetium 99m-DTPA clearance) declined by 7% after 2 cycles of ICE and by 38% after nephrectomy; the mean carboplatin dose was reduced 32% after nephrectomy. Mean GFR remained stable after the third ICE cycle. Although urinary β 2-microglobulin excretion increased during therapy, no patient had clinically significant renal tubular dysfunction at the end of treatment. Treatment with ICE, nephrectomy, and radiotherapy significantly reduces GFR, largely as the result of nephrectomy. Adjustment of carboplatin dosage on the basis of GFR and careful monitoring of renal function may alleviate nephrotoxicity.
Surgical tactics in the treatment of malignant renal tumors in childhood
Pediatric Surgery International, 1997
The authors report the treatment results of 89 children with renal tumors (excluding carcinoma) seen from 1988 to 1992, 71 (79.8%) with favorable-histology Wilms' tumor (survival 86%) and 18 (20.2%) with unfavorable histology, including anaplastic nephroblastoma, clear-cell sarcoma, and rhabdoid tumor (survival 61%). Preoperative chemotherapy (ChT) was given to 46 patients (survival 84%); none was given to 43 with either tumors in the 1st year of life, massive hematuria, or tumor growth during ChT (survival 78%). There were 3 tumor ruptures in the latter group compared to 1 in the former group.
The Management of Synchronous Bilateral Wilms Tumor
Annals of Surgery, 2011
Objective-To provide guidelines for future trials, we reviewed the outcomes of children with synchronous bilateral Wilms tumors (BWT) treated on National Wilms Tumor Study-4 (NWTS-4). Methods-NWTS-4 enrolled 3,335 patients (pts) including 188 pts with BWT (5.6%). Treatment and outcome data were collected. Results-Among 188 BWT pts registered with NWTS-4, 195 kidneys in 123 patients had initial open biopsy, 44 kidneys in 31 pts had needle biopsies. Although pre-resection chemotherapy was recommended, 87 kidneys in 83 pts were managed with primary resection: Complete nephrectomy 48 in 48 pts, 31 partial/wedge nephrectomies in 27 pts, enucleations 8 in 8 pts. No initial surgery was performed in 45 kidneys in 43 pts, 5 kidneys in 3 pts not coded. Anaplasia was diagnosed after completion of the initial course of chemotherapy in 14 pts (initial surgical procedure: 9 open biopsies, 4 needle biopsies, 1 partial nephrectomy). The average number of days from the start of chemotherapy to diagnosis of anaplasia was 390 (range 44-1,925 days). Relapse or progression of disease occurred in 54 children. End stage renal failure occurred in 23 children, 6 of whom had bilateral nephrectomies. The 8 year event free survival (EFS) for BWT with favorable histology was 74%, and overall survival (OS) was 89%; while the EFS for BWT with unfavorable histology was 40%, OS was 45%. Conclusion-The current analysis of patients with BWT treated on NWTS-4 shows that preservation of renal parenchyma is possible in many pts following initial preoperative chemotherapy. The incidence of end-stage renal disease remains significantly higher in children with BWT. Future studies are warranted to address the need for earlier biopsy in non-responsive tumors and earlier definitive surgery to recognize unfavorable histology in these high risk patients.
Nephron-sparing surgery for bilateral Wilms′ tumours: A single-centre experience with 23 cases
African Journal of Paediatric Surgery, 2011
Introduction: The challenge of management with bilateral Wilms' tumours is the eradication of the neoplasm, while at the same time preserving renal function. Surgical management with a variety of nephron-sparing techniques, combined with chemotherapy and occasionally supplemented by transplantation has evolved over the last 30 years to achieve remarkable success. We document the experience of a single centre in a developing country. Material and Methods: Twenty-three bilateral Wilms' tumours were seen in our service between 1981 and 2007. Treatment was, in most cases, according to National Wilms' Tumour Study Group protocols, with initial bilateral biopsy, neoadjuvant chemotherapy, and tumourectomy. Technique of nephrectomy included full mobilization of the tumour-involved kidney, topical cooling with slush ice, vascular exclusion, tumour resection and reconstruction of the remnant kidney. Results: Twelve patients are alive and free of disease one to 15 years after treatment, all with well-preserved renal function (lowest glomerular filtration rate was 65 ml/min per (1.73 m 2). None of the survivors have hypertension. Eleven have died (two of unrelated disease) including six of the seven with spread outside the kidney. All three with unfavourable histology are alive. Four of the five metachronous presentations are alive, as are eight of 12 patients with synchronous bilateral tumours who presented since 2000. Conclusions: Appropriate chemotherapy and nephron-sparing surgery can achieve good results with preservation of adequate renal function in nearly all cases. Unfavourable histology did not have a reduced survival in our series. Metastatic spread outside the kidney had a poor prognosis.