National Wilms Tumor Study Group Research Articles

Update on Wilms tumor

Background: Wilms' tumor is the most common childhood renal neoplasm. Among the worst prognosis forms are the bilateral form. The symptom complex is non-specific for malignancy; the first symptoms may include hypertension. Hematuria occurs in 30% of patients. The incidence of distant metastases at the time of diagnosis in children with Wilms' tumor is estimated to be about 20%.The purpose of this article is to present a review of the literature on the standardization of diagnosis, the latest treatment standards and to assess the prognosis of Wilms' Tumor.
 Matherial and methods: Using PubMed, SCOPUS and Web of Science databases, the authors reviewed the peer-reviewed international literature from 1980-2023 using the keywords: "Wilms tumor," "renal tumor," "nephroblastoma." 
 Discussion: Bilateral tumors have the worst prognosis - according to the National Wilms Tumor Study Group, long-term survival is achieved in only 12% to 56% of patients. The prognosis of Wilms' tumor is also dependent on the occurrence of recurrences - local as well as localized outside the renal tissue. A factor directly affecting long-term survival is recurrence, mainly observed within 24 months after the end of therapy. The limitations of conventional therapies, including surgery, chemotherapy and radiotherapy, in preventing recurrence in WT patients and their potential to cause long-term side effects.
 Conclusions:
 Bilateral Wilms tumor is associated with more aggressive therapy than unilateral disease.In single kidney disease, therapeutic management is aimed at radical removal of all tumor foci, whereas in bilateral tumor, the goal of any management should additionally be to try to preserve the best possible renal function.
 Screening in children is important in cancer. General physicians should refer patients for additional imaging studies when there are any diagnostic doubts, as cancer is characterized by a long, asymptomatic development.

A rare paediatric renal tumour, clear cell sarcoma of kidney

Clear cell sarcoma of kidney is a rare paediatric malignant renal neoplasm which affects children in the 2nd and 3rd year of life. It has a propensity to metastasize to bones, lungs and brain and has a tendency to recur after treatment. The renal biopsy showed classic pattern of clear cell sarcoma of kidney confirmed with immunohistochemical markers and the subsequent post neoadjuvant nephrectomy showed predominantly myxoid pattern possibly due to treatment effects. Since the tumour mimics other paediatric malignant neoplasms with small round blue cell morphology, immunohistochemical markers play a prime role in differentiating them for effective treatment with doxorubicin. Due to aggressive nature and poor prognostic features of clear cell sarcoma of the kidney, it is important to diagnose this tumour and exclude the common neoplasms occurring at this age group prior to neoadjuvant treatment. 1.Argani P, Perlman EJ, Breslow NE, et al. Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center. Am J Surg Pathol 2000; 24: 4–18.2.Ooms AHAG, Vujanić GM, D'Hooghe E, et al. Renal tumors of childhood-a histopathologic pattern-based diagnostic approach. Cancers 2020; 12: 729.3.Kao YC, Sung YS, Zhang L, et al. BCOR overexpression is a highly sensitive marker in round cell sarcomas with BCOR genetic abnormalities. Am J Surg Pathol 2016; 40: 1670–8.

The Clinical Characteristics and Survival Profiles of Wilms Tumor in the United Arab Emirates: A Single-center Retrospective Analysis

Background: Wilms Tumor (WT) is the most common pediatric kidney tumor, yet the disease has variable clinical characteristics and prognostic outcomes across different populations. Objectives: This study aims to review the clinical characteristics and survival outcomes of children with WT who have received treatment at Dubai Hospital, UAE. Methods: A retrospective study was conducted involving the medical records of confirmed WT children (aged < 14 years) who had received treatment between 2013 and 2018. Diagnosis should have been established based on the histopathological examination of operable tumors and needle biopsy for inoperable tumors. The Société Internationale d’Oncologie Pédiatrique (SIOP) WT 2001 protocol was used for patient management, and the UMBRELLA SIOP–RTSG 2016 Wilms tumor pathology and molecular biology protocol was used for histopathologic classification and clinical staging. The presenting sign and symptoms, the findings of diagnostic imaging techniques, histological grading, the received treatments, and follow-up outcomes were collected and analyzed. Kaplan-Meier survival analysis was used to carry out the survival analysis. Results: Ten children were diagnosed with WT (median age of 3.40 years, 60% males). All patients presented with abdominal masses without prominent pain. Synchronous bilateral lesions were found at diagnosis in one patient and metastatic lesions in three patients. Four children were discharged against medical advice; five received treatment according to the SIOP WT 2001 regimen, while the remaining patient was managed in the United States according to the National Wilms Tumor Study Group (NWTSG) protocol. The overall 6-year and relapse-free survival rates were 90% and 80%, respectively. Conclusion: The clinical characteristics and managemental outcomes of children presenting with WT are promising, possibly owing to adopting the SIOP protocol. Considering the small sample size, more large-scale, nation-wide studies are warranted.

Bilateral Wilms tumor with neonatal onset and the importance of prenatal diagnosis

Wilms tumor (WT) occurs in 1:10.000 live births and affects mainly the unilateral kidney. The National Wilms Tumor Study Group registered 6832 patients with WT from 1969 to January 1993, out of whom only 11 patients (0.16%) were newborns. Since 1969, 14 cases of prenatal diagnosis of WT have been reported in literature, two of which were bilateral WT. Currently, the patients’ survival rate is greater than 90%, owing to a combination of surgery, chemotherapy, and radiotherapy for high-risk patients. We report a case of bilateral WT, undiagnosed during the prenatal period due to the incomplete evaluation of the abdomen with ultrasonography. The newborn was vaginally delivered at 40 weeks’ gestational age with a good perinatal adaptation. Suddenly, during the night the newborn showed respiratory distress, bradycardia and then respiratory arrest. For this reason, he was ventilated, intubated and subjected to conventional mechanical ventilation. Despite the normal cardiac ultrasonography, the health care providers suspected a cyanotic congenital heart disease with duct dependency. After a gradual resumption of the oximetry and blood pressure, the infant was transported by the neonatal emergency transport system to the Cardiac Pediatric Surgery Department of a level III hospital. There, the clinical condition became extremely serious and the infant died of asystole. During autopsy, two large tumoral masses were found in both kidneys, also the characterization of the tumors was done through histological exam, which confirmed the diagnosis of WT. A prenatal diagnosis of WT is very important because the families can take advantage of prenatal counseling to understand the risks of continuing the pregnancy and to evaluate the need for abortion, while health care providers can prepare to face a difficult delivery. The review of the literature suggests that prenatal diagnosis of bilateral WT is possible. During prenatal age, the complete assessment of the abdomen with ultrasonography and Eco-Color-Doppler can reveal kidney anomalies and raise suspicion of diseases with urinary malformations.

A Ten Year Retrospective Audit of the Clinic-pathological Management and Treatment Outcomes of Patient’s with Nephroblastoma (Wilms Tumor) at Kenyatta National Hospital

<b>Background and aims: </b>Treatment of Nephroblastoma (Wilms’ tumor) in children is one of medicine's success stories. Due to improvements in surgical techniques, drug therapies, and radiation, 85 to 90 percent of children with Wilms tumor who receive state-of-the-art treatment are cured. While this is true for developed countries the outcomes of nephroblastoma (Wilms’ tumor, WT) in our hospital and other developing countries are notably below those in these countries. There are continuous reviews and updates of clinical pathologic features, treatment designs, and treatment outcomes every 2 or 3 years, in the developed countries. However, in resource poor countries, such frequent reviews and updates are presently not affordable but, nevertheless, efforts should be made to undertake them every 5 or so years, to improve patient management and treatment outcomes. The objectives of this study were to review the clinical presentation and management of children with Wilms tumor and the factors influencing the outcome at Kenyatta National Referral and Teaching Hospital (KNH). This was a retrospective descriptive cross-sectional study. Analysis of the data was carried out using the statistical package for the social sciences (SPSS) software. The findings of the study will form a basis for revision of treatment design for patients diagnosed with Nephroblastoma at KNH, and by extension, other tertiary public health care facilities in Kenya. <b>Patients and methods: </b>The records of 140 WT patients, aged less than 16 years, who were treated in Kenyatta National Hospital, Kenya, during the period from January 1997 to December 2008 were reviewed. The management protocol followed the scheme of the US National Wilms Tumor Study Group (NSWTG). <b>Results: </b>Thirteen cases (38.2%) were diagnosed as stage I, 4 (11.8%) as stage II, 13 (38.2%) as stage III and 2 (5.9%) as stage IV. Eight cases had bilateral disease (stage V). Four-year overall survival (OS) and event free survival (EFS) rates were xx% and xx%, respectively. Univariate analysis by Log-rank test revealed statistically significant associations between OS and nodal status (p- value < 0.01), manifestation of gross hematuria (p-value 0.02), and tumor size of 10 centimeters or more (p-value 0.02). Multivariate analysis found only the nodal status to be independently associated with OS at a Hazard Ratio of 16.6 (p-value < 0.01). Eight of 13 stage I cases and 6/13 stage III cases had relapsed, with two-year post-relapse survival of 42.8%. Significantly poorer outcome was found in cases with early relapse within 200 days after enrollment (p-value 0.02). <b>Conclusion: </b>Childhood Wilms’ tumor presents late in our setting with its consequent management challenges. The need to educate the populace and the primary healthcare providers on the benefits of early diagnosis and treatment of this condition cannot be overemphasized. Large tumor size and gross hematuria were associated with risk of a poorer outcome.

Outcome of patients with intracranial relapse enrolled on national Wilms Tumor Study Group clinical trials.

The occurrence of brain metastases (at diagnosis or at relapse) in patients with Wilms tumor is very rare. We retrospectively reviewed the clinical characteristics of patients with Wilms tumor and relapse to the brain enrolled on the National Wilms Tumor Studies (NWTSs) 1-5. Intracranial relapse was documented in 47 patients (0.5%). Of the 45 patients with adequate data, 26 (58%) patients were male. Thirty-eight (84%) patients had favorable histology Wilms tumor. In 30 patients (67%), the appearance of intracranial disease was preceded by relapse at another site. Ten patients did not have any disease-directed therapy. Surgical resection was attempted in 15 patients; gross total resection was achieved in 11 patients. Twenty-nine patients received brain irradiation; the median dose was 3,000 cGy (range 1,080-4,000 cGy). Twenty-seven patients received chemotherapy. The 5-year overall survival from the time of intracranial relapse was 28.7% (95% confidence interval: 14.4-43.1%). Nine patients (all favorable histology Wilms tumor) were alive with a median follow-up from brain relapse of 140 months (range 35-381 months). All nine survivors received radiation therapy, eight received chemotherapy, and four underwent surgery (two gross total resection, two partial resection). The overall survival after brain metastases of the NWTS-5 patients was significantly higher than the overall survival of the NWTS 1-4 patients (P value = 0.029, log-rank test). Patients with Wilms tumor recurrence involving the brain may have durable survival, particularly those treated in recent years. Multimodality therapy including radiation and chemotherapy should be considered for these patients.

Diagnostic utility of Wilms' tumour-1 protein (WT-1) immunostaining in paediatric renal tumours.

Background & objectives:Renal tumours constitute about 7 per cent of all neoplasms in children. It is important to differentiate Wilms’ tumour (commonest tumour) from non-Wilms’ tumours. The aim of this study was to evaluate the immunoexpression and diagnostic role of Wilms’ tumour-1 protein (WT1) in paediatric renal tumours.Methods:A total of 53 cases of renal tumours in children (below 18 yr) who underwent total nephrectomy were included in this retrospective study. WT1 immunostaining was done using mouse monoclonal WT1 antibody (clone: 6F-H2).Results:Of the 53 cases, 38 (72%) were of Wilms’ tumour. Non-Wilms’ group (15) included six cases of mesoblastic nephroma (MN), two each of clear cell sarcoma (CCSK), renal cell carcinoma (RCC) and peripheral neuroectodermal tumour (PNET) and one each of angiomyolipoma (AML), rhabdomyosarcoma (RMS) and malignant rhabdoid tumour (MRT). Proportion of WT1 positivity in Wilms’ tumour was 100 per cent in contrast to 26.7 per cent in non-Wilms’ tumours (P<0.001). Epithelial and blastemal components of Wilms’ tumour showed moderate (2+) nuclear and cytoplasmic staining in 80 (24/30) and 75 per cent (24/32) cases, respectively. MN, PNET, CCSK and AML were negative for WT1. RMS, RCC and MRT showed cytoplasmic staining, strongest in RMS. No significant association was seen between WT1 expression and NWTSG (National Wilms’ Tumor Study Group) stage.Interpretation & conclusions:WT1 helps to differentiate Wilms’ tumour from other paediatric renal tumours. It may help in differentiating the two subgroups of Wilms’ tumour which have distinct molecular pathogenesis and biological behaviour, however, further prospective studies are required for validation of this hypothesis.

Wilms' Tumor in Children : A Single Institution 10-Year Experience

Objective: To evaluate the disease characteristics and treatment outcome of children with wilms’ tumor at King Hussein Medical Center over a period of 10 years. Methods: We conducted a retrospective review of the medical files of children ( < 14 years ) with wilms’ tumor who were managed at King Hussein Medical Center -Jordan in the interval between June, 2000 until June, 2010 . Patients’ and disease characteristics, treatment modalities and outcome were analyzed. Staging and histopathological classification were preformed according to the system of the National Wilms Tumor Study Group (NWTSG). Descriptive analysis using frequencies was used to describe the study variables. Results: A total of 61 patients (54.1% female) with wilms’ tumor with a median age of 40 months were identified. Their ages ranged between seven months and 7.8 years. The stage frequencies of our cases were : stage I ( 27.9 % ) , stage II (34.4 % ) , stage III ( 16.4 % ) , stage IV ( 11.5 %) and stage V ( 9.8 %) . The 3- year relapse-free survival rate was 100%, 81%, 70%, 57.1% and 83.3% for stages I , II , III , IV and V, respectively. Favorable histology accounted for 86.9 % (53 cases) of our patients . The 3-year relapse-free survival rate was 84.9% for the favorable histology and 62.5% for the anaplastic histology .The 3- year relapse-free survival rate for the entire group was 82%. Conclusion: Combined modality management including surgery , chemotherapy and radiotherapy resulted in satisfactory loco-regional and systemic control and better relapse-free survival in pediatric patients with wilms’ tumor . Stage and histopathologic classification of the disease were important as prognostic factors.

Preoperative chemotherapy and local stage III in nephroblastoma.

The evolution of Nephroblastoma (WT) treatment over the last decades has been one major success around the world. Despite pursuing different upfront treatment approaches the Children's Oncology Group (COG, former National Wilms Tumor Study Group, NWTSG) and the International Society of Paediatric Oncology's Renal Tumor Study Group (SIOP-RTSG) show the same outcome. Treatment starts with preoperative chemotherapy in SIOP-RTSG compared to initial surgery in COG. Response to chemotherapy can be used as a stratification parameter. This allows treating patients with blastemal subtype more aggressively resulting in a better event free survival (EFS). Moreover the percentage of patients with local stage III is less in SIOP-RTSG than in COG studies. Lymph node involvement, in NWTS 5 together with residual microscopic disease, results in a lower EFS in both study groups. But overall survival (OS) is not different comparing patients with or without positive lymph nodes (LN). No other reason for stage III has a significant impact on outcome. The role of radiotherapy for local tumor control in stage III is important, but the radiation dose needs to be questioned as 10.8 Gy used in COG is as efficient as 15 Gy in SIOP-RTSG protocols. In addition in part of low income countries radiotherapy can not be given due to a lack of radiation facilities. Nevertheless some patients are cured without irradiation. The analysis of local stage III patients underlines the importance of preoperative chemotherapy and the need for molecular studies to better stratify patients according to their individual risk.

Clinical efficacy and prognosis with advanced Wilms' tumor in children

Objective To analyze the clinical data of 12 children with advanced Wlims' tumor in children from Feb. 2009 to Jun. 2012. All cases were diagnosed by pathology and to analysis the clinic efficacy and treatment experience were analyzed. Methods Of 12 patients, 10 cases were male and 2 cases were female. The medium age of 12 patients was 2.54 years old(9 months-15 years old). According to pathological stage and clinical stage of The Natio-nal Wilms' Tumor Study Group(NWTSG), 5 cases belonged to stage Ⅲ, and 7 cases stage Ⅳ. Six cases were well-diffe-rentiated tissue type, and 6 cases were poorly differentiated tissue type according to NWTSG. In all patients, different ways of chemotherapy and radiotherapy were selected according to clinical stage and tissue type differentiation. If a patient had repeated recurrence after common surgery and chemotherapy, would treated by autologous peripheral blood stem cell transplantation(APBSCT). Statistic analysis was used to analyze the clinical characters and efficacy and prognosis for 12 patients. Results 1. Initial symptoms: in 12 cases, 8 cases presented abdominal mass(66.6%), 2 cases with abdominal pain and fever(12.7%), and 2 cases with hematuria(12.7%).2. Eleven cases followed up to Jan. 2013, the medium time was 31.5 months(8-131 months). Of 12 cases, 1 case give up therapy and follow-up and 11 cases were followed up. Of those 11 cases followed-up, 4 cases had complete remission(CR), and 1 case had remission in part(PR), the conditions of 5 cases were progressively worse, 1 case replapsed, and 4 patients died. Total survival rate was 63.63%(7/11 cases), and mortality was 36.37%(4/11 cases), and free survival rate was 36.37%(4/11 cases), of that, 1 patient of stage Ⅳ, relapsed 3 times after common radiotherapy and chemotherapy, achieved complete remission after high dose chemotherapy(Melphalan+Carboplatin+Etoposide, CEM) and APBSCT. The estimated 3-year-survival rate was 51.4%. Conclusions The prognosis of advanced Wilms' tumor is poor, and the mortality is still high. High dose chemotherapy with APBSCT may be a valuable method for advanced cases. Key words: Wilms'tumor; Chemotherapy; Autologous peripheral blood stem cell; Transplantation; Child

Astrocyte elevated gene-1 overexpression in histologically favorable Wilms tumor is related to poor prognosis

ObjectiveAstrocyte elevated gene-1 (AEG-1) is associated with tumorigenesis and progression in various types of human cancers. However, the status of AEG-1 expression and its significance in Wilms tumor are still unclear. In this study, we investigated the expression of AEG-1 and evaluated its clinical and prognostic significance in favorable-histology Wilms tumor (FHWT). Materials and methodsImmunohistochemistry was performed to examine AEG-1 protein expression in paraffin-embedded tissues from 38 FHWT patients. All patients underwent radical nephrectomy from January 2003 to June 2008 with subsequent therapy according to National Wilms Tumor Study Group protocols. Statistical analyses were performed to evaluate the association between AEG-1 expression and clinical parameters. ResultsWe found high AEG-1 expression in 17 of 38 (44.7%) patients. AEG-1 expression was significantly correlated with clinical stage (p = 0.019) and status of recurrence (p = 0.023). Importantly, patients with high AEG-1 expression had a shorter disease-free survival and overall survival compared with those with low AEG-1 expression (p = 0.011 and p = 0.013). ConclusionAEG-1 expression is associated with FHWT outcome in this study, and AEG-1 may represent a novel and valuable predictor for prognostic evaluation of FHWT patients.

Outcome of patients with stage II/favorable histology wilms tumor with and without local tumor spill: A report from the National Wilms Tumor Study Group

Intra-operative tumor spill increases the risk of local recurrence of Wilms tumor, and adversely impacts relapse-free (RFS) and overall survival (OS) rates. Surgical checklists, operative notes, institutional pathology reports, central pathology review and flow sheets of 602 patients registered between August 1986 and September 1994 on National Wilms Tumor Study-4 as randomized, followed or switched and coded as Final Stage II, favorable histology (FH) were reviewed. RFS and OS were estimated using the Kaplan-Meier method. Hazard ratios (HRs) were estimated using the Cox model and tested for statistical significance by the log-rank test. Four hundred ninety-nine patients were found after review to have Stage II, FH Wilms tumor. The 8-year RFS percentages were 85.0% (95% confidence interval (CI): 81.1%, 88.1%) for those with no spill compared to 75.7% (65.8%, 83.2%) for those with spill. The 8-year OS percentages were 95.6% (93.1%, 97.3%) for those with no spill compared to 90.3% (82.2%, 94.9%) for those with spill. The HR for relapse among those with spill was 1.55 ((95%CI: 0.97,2.51), P = 0.067) and the HR for death was 1.94 ((0.92,4.09), P = 0.077). RFS and OS were lower for patients who had intra-operative tumor spill. The majority of NWTS Stage II, FH patients with intra-operative tumor spill have an overall excellent outcome when treated with two drug chemotherapy (vincristine and actinomycin D) and no abdominal irradiation.

Image Based Feasibility of Renal Sparing Surgery for Very Low Risk Unilateral Wilms Tumors: A Report from the Children’s Oncology Group

Nephrectomy with lymph node sampling is the recommended treatment for children with unilateral Wilms tumor under the Children's Oncology Group protocols. Using radiological assessment, we determined the feasibility of performing partial nephrectomy in a select group of patients with very low risk unilateral Wilms tumor. We reviewed imaging studies of 60 patients with a mean age of less than 2 years with very low risk unilateral Wilms tumor (mean weight less than 550 gm) to assess the feasibility of partial nephrectomy. We evaluated percentage of salvageable parenchyma, tumor location and anatomical features preventing a nephron sparing approach. A linear relationship exists between tumor weight and computerized tomography estimated tumor volume. Mean tumor weight in the study population was 315 gm. Partial nephrectomy was deemed feasible in only 5 of 60 patients (8%). When considering a select population with very low risk unilateral Wilms tumor (lower volume tumor), only a small percentage of nonpretreated patients are candidates for nephron sparing surgery.

Nephron Sparing Surgery for Unilateral Wilms Tumor in Children with Predisposing Syndromes: Single Center Experience Over 10 Years

Unilateral Wilms tumors associated with predisposing syndromes are treated with preoperative chemotherapy followed by surgical resection. We describe our experience with nephron sparing surgery for Wilms tumor in this population at risk for metachronous lesions. We conducted a retrospective review of all children with a predisposing syndrome who underwent nephrectomy for malignancy during a 10-year period (2000 to 2010). Data collected included age, mode of detection, tumor size, treatment, pathology results, followup time and recurrence episodes. From 2000 to 2010, 13 of 75 (19%) patients treated for Wilms tumor were diagnosed with predisposing syndrome(s). Eight patients with unilateral tumors were treated and had a mean age at diagnosis of 27 months (range 7 months to 9 years). Beckwith-Wiedemann syndrome, isolated hemihyperplasia, WAGR (Wilms tumor, Aniridia, Genitourinary abnormalities, mental Retardation) syndrome and isolated 11p13 deletion were the underlying diagnoses in 3, 2, 2 and 1 patient, respectively. All but 2 patients were diagnosed by screening ultrasound and 5 underwent preoperative chemotherapy. Median tumor size at surgery was 2.5 cm (range 1 to 13). Nephron sparing surgery was performed in 6 of 8 patients. Pathological study showed favorable histology Wilms tumor and nephrogenic rests in 6 and 2 patients, respectively. After a mean followup of 36 months (range 6 to 72) no recurrences were documented and all children had normal creatinine levels. Nephron sparing surgery appears safe for patients with unilateral Wilms tumor associated with predisposing syndrome(s), allowing for the preservation of renal function and good oncologic outcomes for the available followup time. If more studies confirm our observation, current recommendations for the surgical treatment of Wilms tumor may need to reemphasize the value of attempting nephron sparing surgery in this patient population.

Outcomes of Patients With Revised Stage I Clear Cell Sarcoma of Kidney Treated in National Wilms Tumor Studies 1-5

To report the clinical outcomes of children with revised stage I clear cell sarcoma of the kidney (CCSK) using the National Wilms Tumor Study Group (NWTS)-5 staging criteria after multimodality treatment on NWTS 1-5 protocols. All CCSK patients enrolled in the National Wilms Tumor Study Group protocols had their pathology slides reviewed, and only those determined to have revised stage I tumors according to the NWTS-5 staging criteria were included in the present analysis. All patients were treated with multimodality therapy according to the NWTS 1-5 protocols. A total of 53 children were identified as having stage I CCSK. All patients underwent primary surgery with radical nephrectomy. The chemotherapy regimens used were as follows: regimen A, C, F, or EE in 4 children (8%); regimen DD or DD4A in 33 children (62%); regimen J in 4 children (8%); and regimen I in 12 children (22%). Forty-six patients (87%) received flank radiation therapy (RT). Seven children (13%) did not receive flank RT. The median delay between surgery and the initiation of RT was 9 days (range, 3-61). The median RT dose was 10.8 Gy (range, 10-36). The flank RT doses were as follows: 10.5 or 10.8 Gy in 25 patients (47%), 11-19.9 Gy in 2 patients (4%), 20-29.9 Gy in 9 patients (17%), and 30-40 Gy in 10 patients (19%). The median follow-up for the entire group was 17 years (range, 2-36). The relapse-free and cancer-specific survival rate was 100% at the last follow-up examination. The present results have demonstrated that children with revised stage I CCSK using the NWTS-5 staging criteria have excellent survival rates despite the use of varying RT doses and chemotherapy regimens in the NWTS 1-5 protocols.

Wilms Tumor: Preoperative Risk Factors Identified for Intraoperative Tumor Spill

We identified preoperative parameters associated with increased risk of intraoperative Wilms tumor spill. We retrospectively reviewed an institutional database of patients diagnosed with Wilms tumor between 2000 and 2008. Inclusion criteria consisted of available abdominal computerized tomogram and pathological stage I to IV disease. Patient characteristics and neoadjuvant chemotherapy use were noted. After blinding, a radiologist reviewed preoperative computerized tomogram parameters, calculating tumor volume and assigning a preoperative radiological stage. Of 67 patients diagnosed with Wilms tumor 41 (22 males, 19 females) met inclusion criteria, while 26 had incomplete imaging for analysis. Comparison of patients with and without intraoperative tumor spill demonstrated no significant differences in age (3.8 vs 3.6 years), sex (3 males and 3 females vs 19 males and 16 females), body weight or tumor capsule thickness. Preoperative radiological staging was unable to predict pathological stage I to III disease. Six intraoperative tumor spills (15%) were identified (left in 4, right in 2), of which 3 were stage III disease and 3 stage IV. Without neoadjuvant chemotherapy, patients with tumors greater than 1,000 cc had an increased risk of spill (2 of 2 [100%] vs 4 of 33 [12%], p = 0.03). Of 9 patients with stage IV disease 0% (0 of 4) receiving neoadjuvant chemotherapy experienced tumor spill, while lack of neoadjuvant chemotherapy was associated with a 60% (3 of 5 patients, 1 male and 2 females) risk of stage IV spill (p = 0.17). The sole significant tumor spill risk factor identifiable preoperatively was tumor volume greater than 1,000 cc. However, spill occurred at volumes less than 400 cc. Although not statistically significant, neoadjuvant chemotherapy for stage IV disease trended toward diminishing spill risk. Patients with Wilms tumors greater than 1,000 cc may benefit from neoadjuvant chemotherapy with less tumor spill, while stage IV tumors warrant further study in this regard.

Clear Cell Sarcoma of the Kidney

No AccessJournal of UrologyPathology Page1 Jan 2011Clear Cell Sarcoma of the Kidney Katherine E. Watts, Donna E. Hansel, and Gregory T. MacLennan Katherine E. WattsKatherine E. Watts More articles by this author , Donna E. HanselDonna E. Hansel More articles by this author , and Gregory T. MacLennanGregory T. MacLennan More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2010.10.006AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "Clear Cell Sarcoma of the Kidney." The Journal of Urology, 185(1), pp. 279–280 References 1 : Bone metastasizing renal tumor of childhood: histopathological and clinical review of 38 cases. Virchows Arch A Pathol Anat Histol1980; 387: 341. Google Scholar 2 : Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center. Am J Surg Pathol2000; 24: 4. Google Scholar 3 : Genetic and genetic expression analysis of clear cell sarcoma of the kidney. Lab Invest2003; 83: 1293. Google Scholar © 2011 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 185Issue 1January 2011Page: 279-280 Advertisement Copyright & Permissions© 2011 by American Urological Association Education and Research, Inc.MetricsAuthor Information Katherine E. Watts More articles by this author Donna E. Hansel More articles by this author Gregory T. MacLennan More articles by this author Expand All Advertisement PDF downloadLoading ...

Infrequent Tumor Initiative of the Children's Oncology Group: Initial Lessons Learned and Their Impact on Future Plans

The merger of the Pediatric Oncology Group, Children's Cancer Group, the Intergroup Rhabdomyosarcoma Study Group, and the National Wilms Tumor Study Group in 2000 offered the newly formed Children's Oncology Group (COG), an opportunity to study rare cancers that had not been the subject of organized evaluation within the context of a cooperative group. In 2002, the COG formed the rare tumor committee which is comprised of four subcommittees. This article details the experience of the infrequent tumor subcommittee for the period of 2002 to 2007. During the initial implementation of this strategy, we have observed low rates of registration within the COG registry and low levels of participation in open banking, biology, and first-line therapeutic studies. This initial experience has allowed us to develop alternative strategies to increase registration rates and clinical trial enrollments. It is hoped that these new plans will allow us to increase our ability to better understand the biology and improve the treatment outcome of young patients with infrequent cancers. Furthermore, our initial experience has demonstrated to us the potential power of expanded cooperation and collaboration at a global level.

Nephrectomy‐only for Wilms tumour: Negotiating the tangled web requires multi‐professional input

Nephrectomy‐only for Wilms tumour: Negotiating the tangled web requires multi‐professional input

219 How to manage the patient who presents with stage IV colorectal cancer. The role of the colorectal surgeon

Wilms' tumor, or nephroblastoma, is the most common malignant renal tumor of childhood. It occurs with an annual incidence of 7 cases per million children aged less than 15 years.9 Approximately 450 new cases are diagnosed each year in North America. Although at the beginning of this century, most children with Wilms' tumor died of the disease, survival now exceeds 80%.26 This tremendous success story in childhood cancer was facilitated initially by individuals. Subsequent advances were achieved by institutions and cooperative groups. In the past 3 decades, the National Wilms' Tumor Study Group (NWTSG) and the International Society of Paediatric Oncology (SIOP) have contributed to improving the clinical management of children affected by Wilms' tumor. The NWTSG has studied most patients with Wilms' tumor in North America in randomized trials since 1969 and has collected data on more than 6000 patients. Based on a strategy of immediate nephrectomy, the NWTSG recommends early surgical resection of the tumor and affected kidney.35 Subsequently, depending on the histology of the tumor and the clinicopathologic stage, patients should receive chemotherapy. Patients with advanced disease or specific adverse prognostic features should receive additional radiotherapy. In contrast, the SIOP approach recommends preoperative chemotherapy for all tumors, with postoperative treatment for localized tumors dictated according to the stage assigned at the time of surgery.33Although it is a rare disease, Wilms' tumor is one of the childhood cancers that have provided significant information regarding the genetic events leading to the development of cancer in general. Wilms' tumors do not display as much genetic instability69 as adult cancers; hence, the majority of genetic alterations observed are likely to have a role in their development. The systematic study of Wilms' tumor occurring in association with congenital abnormalities has increased understanding of the pathogenesis of this childhood cancer. For example, WT1, the first Wilms' tumor suppressor gene at chromosome 11p13, was identified as a direct result of the study of children with Wilms' tumor who also had aniridia, mental retardation, and genitourinary anomalies. Karyotypic analysis of these children revealed a deletion within the short arm of one copy of chromosome 11.87 Study of the deleted region at band p13 led to the cloning of WT1 in 1990.11, 17, 32 This gene encodes a transcription factor critical to normal kidney and gonadal development.59 Similarly, the study of patients with Wilms' tumor and Beckwith-Wiedemann syndrome, another congenital Wilms' tumor predisposition syndrome, implicated the existence of a second Wilms' tumor suppressor gene, designated WT2. The existence of additional genetic loci involved in the development of Wilms' tumor is suggested by linkage analyses in large pedigrees with familial transmission of susceptibility to Wilms' tumor.70, 84 Loci at 16q,20, 38, 69 1p,38 7p,72, 88, 98 and 17p5, 64 have also been evoked in the biology of Wilms' tumor, although these loci do not predispose to Wilms' tumor but are associated with phenotype or outcome.This article reviews the most important known genetic loci involved in the biology of Wilms' tumor.