National Wilms Tumor Study Group Research Articles

Bilateral Wilms' tumors: a single-center experience with 19 cases

Background/Purpose Bilateral Wilms' tumors present a therapeutic challenge, particularly with delay in presentation, when there is poor response to chemotherapy and when associated with nephroblastomatosis. The primary aim of management is eradication of neoplasm, while at the same time preserving of renal function. Methods Nineteen bilateral Wilms' tumors were seen in our service between 1981 and 2003. This represented 10% of the 190 patients with Wilms' tumors seen during this period. There were 12 female and 7 male patients ranging in age from 7 months to 8 years. Fourteen had synchronous presentation, one of whom had liver metastasis at diagnosis. Of the 5 patients with metachronous tumors, 3 had their initial nephrectomies done elsewhere. Nephroblastomatosis was identified in 18 (95%) of the patients. Treatment was, in most cases, according to National Wilms Tumor Study Group protocols, with initial bilateral biopsy, neoadjuvant chemotherapy, and tumorectomy. Where indicated, nephrectomy (partial or complete) involved using ice dam topical cooling and vascular control, and in one case, bench surgery and extensive renal reconstruction with orthotopic autotransplantation. Revision tumorectomy was used on 3 occasions for recurrence in areas of nephroblastomatosis. There were 6 extrarenal relapses. Results Ten patients are alive and free of disease 1 to 15 years after treatment, all with well preserved renal function (lowest recorded glomerular filtration rate was 85 mL/min per 1.73 m 2). Nine have died (2 of unrelated disease), including 6 of the 7 with spread outside the kidney. All 3 with unfavorable histology are alive. Of the 5 with metachronous presentations, 4 are alive, as are 7 of 10 who presented in the last decade. Conclusions Appropriate chemotherapy and conservative nephron-sparing and innovative surgery can achieve good results with preservation of adequate renal function in nearly all cases. Nephroblastomatosis was an almost universal finding and requires close monitoring because Wilms' tumors developed in residual suspect areas. Revision surgery was effective. Unfavorable histology did not have a reduced survival in our series. Metastatic spread outside the kidney had a poor prognosis.

Treatment of anaplastic Wilms tumor: A report from the National Wilms Tumor Study Group

8508 Background: The prognosis for children with anaplastic Wilms tumor (AWT) is poorer than that for children with favorable histology Wilms tumor. An objective of the fifth National Wilms Tumor Study (NWTS-5) was to evaluate the efficacy of treatment regimens for AWT. Methods: Prospective single-arm studies were conducted between August 1995 and June 2002. Patients with stage I AWT were treated with Regimen EE-4A (vincristine/dactinomycin x 18 weeks). Patients with stages II-IV focal anaplasia (FA) were treated with Regimen DD-4A (vincristine/dactinomycin/doxorubicin x 24 weeks and XRT). Patients with stages II-IV diffuse anaplasia (DA) were treated with Regimen I (vincristine/doxorubicin/cyclophosphamide alternating with cyclophosphamide/etoposide x 24 weeks and XRT). Results: 282 patients with AWT enrolled on NWTS-5. 79 of these patients had major protocol violations, mostly consisting of late regimen changes due to revision of histologic subtype after central pathology review. Among the 203 patients ...

Treatment of Wilms tumor relapsing after initial treatment with vincristine, actinomycin D and doxorubicin. A report from the National Wilms Tumor Study (NWTS) Group

8507 NWTS - 5 was a multi-institutional clinical trial for patients less than 16 years of age at diagnosis with specific renal neoplasms who were diagnosed between August 1, 1995 and May 31, 2002. A uniform approach to the treatment of patients with relapse was employed. 103 patients who relapsed after initial chemotherapy with vincristine, actinomycin D and doxorubicin, and radiation therapy were registered on stratum C of the NWTS-5 Relapse protocol. Twelve patients were not evaluable: 5 due to insufficient data, 6 due to major protocol violations and one for refusal of therapy. Among the 91 remaining patients, 14 with bilateral Wilms tumor at diagnosis, 1 who developed a contralateral relapse, and16 who did not achieve a complete response to the initial therapy were not included in this analysis. Relapse treatment included initial chemotherapy, followed by surgical excision, if feasible, and radiation therapy. Maintenance chemotherapy included alternating courses of the drug pairs of cyclophosphamide and etoposide and carboplatinum and etoposide. The median duration of follow-up of surviving patients was 3.44 years from registration on the relapse protocol. The most frequent toxicity was profound thrombocytopenia that contributed to one on-study death, and discontinuation of maintenance therapy for 12 patients. One patient developed acute myelogenous leukemia. These results demonstrate that approximately one-half of children with Wilms tumor who relapse after initial treatment with vincristine, actinomycin D, doxorubicin and radiation therapy can be successfully re-treated. Author Disclosure Site Number of Patients Number of Second Events 4-year EFS (%) Number of Deaths 4-year Survival (%) All 60 33 42.3 27 48.0 Operative bed lung other 7 6 14.3 4 38.1 Liver other 6 5 16.7 4 33.3 Abdomen or pelvis lung 6 5 16.7 5 0.0 Lung only 33 16 48.9 13 52.8 Lung and other 6 1 80.0 1 80.0 Other 2 0 - 0 - No significant financial relationships to disclose.

Treatment of Wilms tumor relapsing after initial treatment with vincristine and actinomycin D. A report from the National Wilms Tumor Study (NWTS) Group

8551 NWTS - 5 was a multi-institutional clinical trial for patients less than 16 years of age at diagnosis with specific renal neoplasms who were diagnosed between August 1, 1995 and May 31, 2002. A uniform approach to the treatment of patients with relapse was employed. 72 patients who relapsed after immediate nephrectomy (stages I and II), initial chemotherapy with vincristine and actinomycin D and no radiation therapy were registered on stratum B of the NWTS-5 Relapse protocol. Four patients were not evaluable: one due to insufficient data and 3 due to major protocol violations. Among the 68 remaining patients, 5 with bilateral Wilms tumor at diagnosis, 3 who developed a contralateral relapse and 1 with persistent disease were not included in this analysis. Relapse treatment included surgical excision, when feasible, radiation therapy and alternating courses of vincristine, doxorubicin and cyclophosphamide and etoposide and cyclophosphamide. The most frequent toxicities were hematological. These results demonstrate that a significant proportion of children with Wilms tumor who relapse after initial treatment with vincristine and actinomycin D can be successfully re-treated. Author Disclosure Results by Site of Relapse Site of Relapse Number of Patients Number of Second Events 4-year EFS (%) Number of Deaths 4-year Survival (%) All 59 15 69.1 10 82.2 Operative bed lung other sites 18 2 77.8 2 88.0 Liver 1 1 0.0 0 0.0 Abdomen or pelvis other sites 6 1 66.7 0 0.0 Lung only 31 9 67.8 7 81.0 Lung and other 2 1 50.0 1 50.0 Other 1 0 - 0 - No significant financial relationships to disclose.

CT-Estimated Volume of Wilms Tumor Can Predict Weight

Wilms tumor weight was used to recruit patients in a recent National Wilms Tumor Study (NWTS) group trial. The authors hypothesized that a simple calculation of tumor volume based on a preoperative CT scan could predict tumor weight. The authors reviewed charts and CT images of patients with Wilms tumors who were treated at their institution between 1985 and 2002. Tumor volume was calculated as: V = 1/6pi x d (long axis) x d (short axis) x d (craniocaudal). Weight and calculated tumor volume were correlated using linear regression. Complete data of tumor weight and volume could be determined in 25 of the 49 patients. These were highly correlated (Spearman R = 0.97). Wilms tumor weight can be predicted based on a simple estimate of tumor volume on a preoperative CT scan. CT-estimated volume may replace weight as a prognostic factor and in guiding management.

Pediatric Renal Tumors: Practical Updates for the Pathologist

Pediatric renal tumors were targeted by the National Wilms Tumor Study Group for 4 decades with extraordinary success. Within this historic context, this review provides a summary of the new Children's Oncology Group renal tumor protocols that will be opening in the very near future, focusing on their pathologic requirements. All renal tumors must first be registered on the Renal Tumor Classification and Banking Protocol, followed by registration on 1 of 4 primary therapeutic protocols based on histology, stage, and molecular analysis. This requires prompt submission of samples for molecular analysis and central pathologic review. Changes in staging criteria include classification of all tumor spillage as stage III, and requirement of regional lymph node evaluation for eligibility for stage I Wilms tumors (WTs) weighing less than 550 g in infants younger than 24 months and for stage I clear cell sarcoma. Patients with unilateral favorable histology WT with loss of heterozygosity for chromosomes 1p and 16q will receive more aggressive chemotherapy at each stage. Patients with bilateral WT and patients with diffuse hyperplastic perilobar nephroblastomatosis will be eligible for a novel therapeutic protocol requiring pathologic classification based on response of tumor to previous therapy. Stage I anaplastic WT will be targeted with more aggressive chemotherapy than in the past. For the first time, pediatric renal cell carcinoma will be eligible for a cooperative group protocol. All rhabdoid tumors outside the central nervous system will be eligible for a single protocol. In conclusion, these new protocols bring considerable change in their overall organization, in eligibility, and in therapy.

Introduction to the Special Issue: Surviving Pediatric Cancer: Research Gains and Goals

Nearly every review of the research literature in pediatric oncology describes the significant progress of medical treatment over the past 30 years resulting in the increased survival rate for children and adolescents with cancer. Concurrent with these medical advances, a vast amount of research has emerged in an area that has come to be known as “pediatric psycho-oncology.” From these studies we have learned a great deal about the neurocognitive and psychosocial functioning of children, adolescents, and young adults with cancer and their families. The field has progressed from strictly descriptive studies of adjustment to more explanatory research and to development of interventions to improve functioning. It is an apt time for an overview of the progress made, the current state of the science, and the perspectives on future directions. We believe that such a review can offer lessons that will benefit not only the researchers who work in pediatric oncology but also those who work with children coping with other lifethreatening and chronic diseases. The impetus for this special issue began with the merger of the two largest cooperative clinical trials groups in pediatric oncology—the Pediatric Oncology Group and the Children’s Cancer Group—as well as two disease-focused groups: the National Wilms Tumor Study Group and the International Rhabdomyosarcoma Study Group. Many psychologists have been actively involved in these groups’ research studies and have come together to form the psychology discipline of the new group, the Childhood Cancer Group (COG). As coprincipal investigators, we were pleased to receive funding from the COG and the National Cancer Institute to develop a research summit on the state of the science in pediatric psycho-oncology, which was held in San Antonio, Texas, October 2001. As a result of the summit and with encouragement from Ron Brown, the editor of this journal, we called for papers that reviewed current literature and that incorporated discussions from the summit. Following peer review, we invited commentaries on the submitted papers—hence, this special issue. As colleagues who have been working in this field over the past 30 years, we have been pleased to edit this special issue and to work with such productive and thoughtful senior and junior investigators to provide a critical overview of the field. Because we wanted to include viewpoints spanning this 30-year period, we invited papers from senior researchers, who began their careers when survival rates were lower and when little was known about coping, adaptation, and neuropsychological functioning; and from junior investigators, whose careers started on the foundation of recent treatment and research advances and who may therefore have different views on current findings and future directions. We believe that the perspectives of these researchers are generally representative of the field at large, but we also encourage readers to review the articles by the many pediatric psycho-oncology authors who are listed in the authors’ extensive bibliographies. The neurocognitive and psychosocial sections are separated in this issue, but their subjects should always be considered intertwining aspects of the child’s total functioning. Although neurocognitive functioning can be considered an endpoint, as it defines the capacities and limitations placed on the child as a result of disease and treatment, it is also an intermediate endpoint. Research often necessitates separating neurocognitive or specific psychosocial variables such as depression; however, it is important to remember that in the lives of cancer survivors, neurocognitive and psychosocial variables are interdependent and that our goal as researchers is to optimize each survivor’s overarching quality of life. In the neurocognitive and psychosocial sections of this issue, the first paper is devoted to an overview of the state of the science and the second paper to current intervention research. The section entitled “Enhancing the Research Environment for the Future” begins with a paper about psychological research in the clinical trials

Decrease in LDL receptor-related protein expression and function correlates with advanced stages of Wilms tumors

The molecular processes responsible for the invasive phenotype of pediatric Wilms tumors (WT) are poorly understood. A candidate WT suppressor gene (WT1) has been found mutated in a number of these pediatric kidney tumors. However, the disruption of normal WT1 protein function cannot solely explain WT growth. The aim of the present study is to identify new molecular players that regulate the invasive character of WT. Fresh frozen samples from 45 renal tumors of Wilms were obtained from the National Wilms Tumor Study Group's Biological Samples Bank. Gelatin zymography, Western blotting, and immunodetection were used to compare tissue biopsies originating from the infiltrating (stage III), metastatic (stage IV), and anaplastic phenotype of Wilms tumors (WT). The expression of the low-density lipoprotein receptor-related protein (LRP) diminished in stage IV and anaplastic WT. Moreover, the expression of RAP, an LRP intracellular chaperone, was also decreased. The diminished expression of LRP and RAP correlated with increased levels of several known extracellular ligands that LRP usually recycles from the extracellular matrix (ECM) environment, including PAI-1, MMP-9, and TIMP-1. The proteolytic processing of MT1-MMP, a functional regulator of LRP, also correlated with the WT invasive phenotype. The low expression of LRP, whose function is regulated by MT1-MMP and whose activity in recycling ECM-associated proteolytic enzymes becomes drastically diminished in advanced stages of WT, may in part explain the acquired invasive potential of the developing WT pediatric cancer.

Treatment outcomes in adults with favorable histologic type Wilms tumor—an update from the National Wilms Tumor Study Group

To describe the clinical outcomes in adults with favorable histologic type (FH) Wilms tumor (WT) registered in the National Wilms Tumor Studies (NWTS) 4-5. We also describe the results of patients treated in the "modern era" (1979-2001) with surgical staging, central pathology review and stage-appropriate multimodality treatment. Twenty-three adult patients (> or =16 years of age) with FHWT after central pathology review were registered on NWTS 4-5. The tumor stage distribution was Stage I in 5, Stage II in 8, Stage III in 6, and Stage IV in 4 patients. All patients underwent primary nephrectomy followed by multiagent chemotherapy and/or radiotherapy (RT). All patients underwent tumor stage-based chemotherapy that generally followed existing NWTS Group (NWTSG) protocols. To analyze the outcomes of adult patients treated in the "modern era," the data from this report were combined with the data from 22 patients with FHWT previously reported in 1990 by the NWTSG. The 5-year relapse-free survival, overall survival, and disease-specific survival (DSS) rate was 77.3%, 82.6%, and 95.7%, respectively, for patients registered in the NWTS 4-5 protocols. Three patients (13%) died of chemotherapy-induced hepatic venoocclusive disease. For a total of 45 adults with FHWTs treated in the "modern era," the overall survival rate was 82%. The survival rate for those with Stage I, II, III, and IV disease was 100%, 92%, 70%, and 73%, respectively. Of the 12 Stage I-II patients treated with two drugs and no RT, the survival rate was 100%. The survival rate for Stage III and IV patients treated with three drugs and RT was 63% and 70%, respectively. The results of this report demonstrate that adults with FHWT treated with a multimodality approach similar to NWTSG protocols have good survival. We recommend that all adult patients be treated with stage-appropriate combined modality therapy, and furthermore, be entered in current Children's Oncology Group WT protocols so that coherent data can be gathered for this relatively rare tumor. Finally, all patients should be monitored for signs and symptoms of hepatic venoocclusive disease.

The treatment of stages I-IV favorable histology Wilms' tumor.

The prognosis for children with Wilms’ tumor (WT) has improved dramatically. During the last three decades, the National Wilms Tumor Study Group (NWTSG), the International Society of Pediatric Oncology (SIOP), and the United Kingdom Children’s Cancer Study Group (UKCCSG) conducted sequential studies of treatments for children with WT. The results will be reviewed in the light of long-term follow-up. Areas for future investigation will be identified.

Wilms tumor screening is unnecessary in Klippel-Trenaunay syndrome.

Children with hemihypertrophy are screened for Wilms tumor, because this condition is a risk factor for developing the neoplasm. Patients with Klippel-Trenaunay syndrome (KTS) are often considered potential candidates for Wilms tumor, because they have unilateral overgrowth of the lower limb. In our experience, however, an association between KTS and Wilms tumor has not been observed. To determine whether KTS and Wilms tumor are associated, we reviewed our institutional experience for patients with both diagnoses and searched the Klippel-Trenaunay literature for patients with Wilms tumor. The National Wilms Tumor Study Group database also was studied to identify patients with KTS. Two-sided exact binomial tests were used to evaluate whether patients with 1 condition had an increased risk for the other. Ninety-five percent confidence intervals for these 2 risks were compared with the general population risks of Wilms tumor (1 in 10 000) and KTS (1 in 47 313). None of the 115 patients with KTS followed at our institution developed Wilms tumor. One case of Wilms tumor has been reported in 1363 patients with KTS in the literature, giving a confidence interval of (1/57 377) and (1/267). None of the 8614 patients in the National Wilms Tumor Study Group database had KTS, giving a confidence interval of (0, 1/2336). Because the risks of KTS and Wilms tumor in the population fall within these confidence intervals, one cannot conclude that the risks of KTS among Wilms tumor patients or Wilms tumor among KTS patients are any different from the corresponding risks in the general population. Patients with KTS are not at increased risk for developing Wilms tumor and thus should not undergo routine ultrasonographic screening.

Characteristics and Outcomes of Children With the Wilms Tumor-Aniridia Syndrome: A Report From the National Wilms Tumor Study Group

Children with the rare Wilms tumor (WT)-aniridia (WAGR) syndrome have not had systematic evaluation of their clinical and pathologic features. We compared demographics, disease characteristics, and treatment outcomes in a large cohort of WT patients who did or did not have the WAGR syndrome. Clinical and pathology records were reviewed for 8,533 patients enrolled between 1969 and 2002 by the National Wilms Tumor Study Group. Sixty-four patients (0.75%) had the WAGR syndrome. For WAGR and non-WAGR patients, respectively, the average birth weights (2.94 and 3.45 kg), median ages at diagnosis (22 and 39 months), and the percentages with bilateral disease (17% and 6%), metastatic disease (2% and 13%), favorable histology (FH) tumors (100% and 92%), and intralobar nephrogenic rests (ILNR; 77% and 22%) all differed. Survival estimates for WAGR and non-WAGR patients were 95% +/- 3% and 92% +/- 0.3% at 4 years but 48% +/- 17% and 86% +/- 1.0%, respectively, at 27 years from diagnosis. Five late deaths in WAGR patients were from end-stage renal disease (ESRD). The excess of bilateral disease, ILNR-associated FH tumors of mixed cell type, and early ages at diagnosis in WAGR patients all fit the known phenotypic spectrum of constitutional deletion of chromosome 11p13. Despite a favorable response of their WT to treatment, WAGR patients have a high risk of ESRD as they approach adulthood. The renal pathology associated with this apparent late manifestation of WT1 deletion, and the explanation for the abnormally low birth weights in patients with del 11p13, have yet to be determined.

Genetic and Genetic Expression Analyses of Clear Cell Sarcoma of the Kidney

Clear cell sarcoma of the kidney (CCSK) represents a significant diagnostic and clinical challenge. In search of diagnostically useful or biologically significant genetic abnormalities, we screened 30 CCSKs from the National Wilms Tumor Study Group. Genetic gains and losses were analyzed using comparative genomic hybridization; loss of heterozygosity at 11p15 was studied using microsatellite analysis. Loss of imprinting (LOI) was studied using allele-specific expression or methylation analysis at the ApaI polymorphic site for IGF2, AluI and RsaI sites for H19, and Cfo I site for SNRPN. Comparative genomic hybridization analysis revealed quantitative abnormalities in only 4 of 30 CCSKs. Two showed gain of 1q, one also showed loss of 10q, and the other also showed loss of terminal 4p. The other two cases demonstrated chromosome 19 loss and chromosome 19p gain, respectively. All 22 cases informative for 11p15 showed retention of both alleles. Of 14 CCSKs informative for IGF2, six showed biallelic expression; all three CCSKs informative for H19 exhibited monoallelic expression. The normal imprint pattern was present in all six CCSKs analyzed for SNRPN methylation. These data demonstrate an absence of consistent genetic gains or losses in CCSKs using these methods. The high frequency of LOI for IGF2 in CCSKs (43%) is comparable to that reported in Wilms tumors. The retention of imprinting at the SNRPN and H19 loci confirm that LOI is not a ubiquitous epigenetic change. This suggests that IGF2, a potent growth factor, may play a role in the development or progression of CCSK.

Outcome of children with cystic partially differentiated nephroblastoma treated with or without chemotherapy

Background/purpose Cystic partially differentiated nephroblastoma (CPDN) is a rare variant of Wilms’ tumor thought to be more favorable than standard nephroblastoma. The purpose of this report is to examine the outcome of children with CPDN, after nephrectomy, treated with vincristine and dactinomycin based chemotherapy (+/− doxorubicin) or no chemotherapy. Methods Patients were registered with the National Wilms’ Tumor Study Group (NWTSG) and data were collected prospectively. All patients had central review by the NWTSG Pathology Center to confirm the diagnosis of CPDN. Results Twenty-one patients were identified with a diagnosis of CPDN. Thirteen patients received chemotherapy, and 8 patients did not. In the chemotherapy group the stage distribution was as follows: stage I (n = 10), stage II (n = 2), stage V (n = 1). In the no-chemotherapy group, all 8 patients were stage I. All patients had complete tumor resection. There were no cases of disease progression or recurrence in any patient. In patients receiving chemotherapy, 30% (n = 4) had toxicities causing dose reduction. Conclusions The outcome of patients with CPDN is favorable with 100% survival rate and no recurrences. For stage I patients, treatment with complete tumor resection appears to be as efficacious as nephrectomy plus chemotherapy. Stage II patients also have excellent outcome when treated with tumor resection and postoperative vincristine and dactinomycin.

The occurrence of Wilms tumor in horseshoe kidneys: A report from the National Wilms Tumor Study Group (NWTSG)

Background/Purpose: An increased incidence of Wilms tumor has been noted in patients with a horseshoe kidney. These represent a difficult diagnostic and therapeutic challenge. The charts of all National Wilms Tumor Study Group (NWTSG) patients with Wilms tumor occurring in a horseshoe kidney were reviewed. Methods: From 1969 to 1998, 8,617 patients were enrolled in the NWTSG. Forty-one patients were found to have a Wilms tumor arising in a horseshoe kidney for an incidence of 0.48%. Their records were reviewed retrospectively. Results: Horseshoe kidney was not recognized preoperatively in 13 patients, 10 of whom were evaluated with computed tomography (CT). Four of the 10 also had renal ultrasonography and one an intravenous pyelogram (IVP). Two of the 13 were evaluated with an IVP only, and the last had no preoperative imaging studies performed. Stage at presentation was stage I, 10 pts; stage II, 10; stage III, 12; stage IV, 6; stage V, 3. Primary surgical resection was performed in 26 patients, including 23 nephrectomies and 3 partial nephrectomies. Fifteen children were treated with preoperative chemotherapy after initial biopsy of the tumor. The mean total remaining renal parenchyma after all operations (excluding treatment of relapses) was approximately 75%. Surgical complications occurred in 14.6% of patients, including 2 urine leaks, 2 ureteral obstructions, and 1 ureteral injury. Two patients had transient renal failure. Conclusions: The diagnosis of horseshoe kidney often was missed on preoperative imaging. Accurate preoperative diagnosis is important in planning the operative approach and may help to decrease complications related to transection of the urinary collecting system. Although 37% of patients with Wilms tumor arising in a horseshoe kidney were judged inoperable at initial exploration, all were amenable to resection after chemotherapy. J Pediatr Surg 37:1134-1137. Copyright 2002, Elsevier Science (USA). All rights reserved.

Characteristics and Outcome of Children With Beckwith-Wiedemann Syndrome and Wilms’ Tumor: A Report From the National Wilms Tumor Study Group

No AccessJournal of UrologyUROLOGICAL SURVEY: Pediatric Urology: Abstracts1 Jul 2002Characteristics and Outcome of Children With Beckwith-Wiedemann Syndrome and Wilms’ Tumor: A Report From the National Wilms Tumor Study Group Douglas A. CanningM.D. Douglas A. CanningDouglas A. Canning More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(05)64942-7AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "Characteristics and Outcome of Children With Beckwith-Wiedemann Syndrome and Wilms’ Tumor: A Report From the National Wilms Tumor Study Group." The Journal of Urology, 168(1), pp. 382–383 © 2002 by American Urological Association, Inc.FiguresReferencesRelatedDetails Volume 168Issue 1July 2002Page: 382-383 Advertisement Copyright & Permissions© 2002 by American Urological Association, Inc.MetricsAuthor Information Douglas A. Canning More articles by this author Expand All Advertisement PDF downloadLoading ...

Characteristics and Outcome of Children With Beckwith-Wiedemann Syndrome and Wilms??? Tumor: A Report From the National Wilms Tumor Study Group

Characteristics and Outcome of Children With Beckwith-Wiedemann Syndrome and Wilms??? Tumor: A Report From the National Wilms Tumor Study Group

Pregnancy outcome after treatment for Wilms tumor: a report from the National Wilms Tumor Study Group.

This study was undertaken to determine the effect, if any, of prior treatment with radiation therapy or chemotherapy for Wilms tumor diagnosed during childhood or adolescence on live births, birthweight, and the frequency of congenital malformations. We reviewed pregnancy outcomes among survivors of Wilms tumor treated with or without irradiation to the flank or tumor bed on National Wilms Tumor Studies 1, 2, 3, and 4 using a maternal questionnaire and review of both maternal and offspring medical records. We received reports regarding 427 pregnancies with duration of 20 weeks or longer, including 409 liveborn singletons for whom 309 sets of medical records were reviewed. Malposition of the fetus and early or threatened labor were more frequent among irradiated women. Both were more frequent among women who received higher radiation therapy doses. The offspring of the irradiated female patients were more likely to weigh less than 2,500 g at birth and to be of less than 36 weeks gestation, with both being more frequent after higher doses of radiation. An increased percentage of offspring of irradiated females had one or more congenital malformations. Women who receive flank radiation therapy as part of their treatment for Wilms tumor are at increased risk of fetal malposition and premature labor. The offspring of these women are at risk for low birthweight, premature (< 36 weeks gestation) birth, and the occurrence of congenital malformations. These risks must be considered in the obstetrical management of female survivors of Wilms tumor.

The spectrum of metanephric adenofibroma and related lesions: clinicopathologic study of 25 cases from the National Wilms Tumor Study Group Pathology Center.

The authors report nine new metanephric adenofibroma (MAFs; previously termed nephrogenic adenofibroma) and 16 related tumors from the files of the National Wilms Tumor Study Group Pathology Center (NWTSGPC). All tumors contained a variable amount of a bland spindle cell stroma, which is essentially identical to the recently described metanephric stromal tumor (MST). Features that distinguish this stroma from congenital mesoblastic nephroma (CMN) include intratumoral angiodysplasia, concentric cuffing of entrapped tubules ("onion skinning"), and heterologous differentiation. The epithelial components of these lesions spanned a wide range of appearances. All tumors contained at least focally an inactive embryonal epithelium identical morphologically to metanephric adenoma (MA), and hence each case could be classified as containing MAF. The epithelium of nine tumors had this appearance throughout, and hence these were considered usual MAFs. The epithelium of four tumors demonstrated increased mitotic activity but was otherwise similar to MA. The epithelial component of seven tumors spanned a morphologic spectrum from inactive MA to malignant epithelial predominant Wilms tumor (WT), with gradual transitions noted in several cases. Five other tumors contained a carcinomatous component distinct from these lesions but identical morphologically to papillary renal cell carcinoma (PRCC). In one of these cases, this component had metastasized to the regional lymph nodes at the time of diagnosis. No tumor recurred during follow-up, although almost all patients received adjuvant therapy for WT regardless of their tumor's histology and NWTSGPC diagnosis. In conclusion, MAF is a biphasic tumor that spans the morphologic spectrum between benign pure stromal (MST) and pure epithelial (MA) lesions, and can merge with the morphology of WT, supporting the concept that these are all related lesions. A relationship to PRCC is also evident.

ADULT CLEAR CELL SARCOMA OF THE KIDNEY WITH ATRIAL TUMOR THROMBUS

No AccessJournal of UrologyCLINICAL UROLOGY: Case Reports1 Mar 2001ADULT CLEAR CELL SARCOMA OF THE KIDNEY WITH ATRIAL TUMOR THROMBUS SAM B. BHAYANI, HELEN LIAPIS, and ADAM S. KIBEL SAM B. BHAYANISAM B. BHAYANI More articles by this author , HELEN LIAPISHELEN LIAPIS More articles by this author , and ADAM S. KIBELADAM S. KIBEL More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(05)66557-3AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "ADULT CLEAR CELL SARCOMA OF THE KIDNEY WITH ATRIAL TUMOR THROMBUS." The Journal of Urology, 165(3), pp. 896–897 References 1 : Clear cell sarcoma of the kidney with tumor extension into the right atrium. J Cardiovasc Surg (Torino)1998; 39: 489. 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 : Clear cell sarcoma of kidney in an adolescent and young adults: a report of four cases with ultrastructural, immunohistochemical, and DNA flow cytometric analysis. Am J Surg Pathol1999; 23: 1455. Google Scholar From the Division of Urology (Surgery) and Department of Pathology, Washington University School of Medicine, St. Louis, Missouri© 2001 by American Urological Association, Inc.FiguresReferencesRelatedDetails Volume 165Issue 3March 2001Page: 896-897 Advertisement Copyright & Permissions© 2001 by American Urological Association, Inc.Keywordsthrombosiskidneysarcoma, clear cellMetricsAuthor Information SAM B. BHAYANI More articles by this author HELEN LIAPIS More articles by this author ADAM S. KIBEL More articles by this author Expand All Advertisement PDF downloadLoading ...