National Cancer Institute®
Last Modified: July 1, 2002
UI - 12051352
AU - Oeberst JL; Barnard JJ; Prahlow JA
TI - Sudden death due to undiagnosed Wilms' tumor in an adult.
SO - J Forensic Sci 2002 May;47(3):638-9
AD - Southwestern Institute of Forensic Sciences and University of Texas Southwestern Medical School, Department of Pathology, Dallas, USA.
Sudden unexpected deaths due to natural causes constitute a large number of cases encountered by the forensic pathologist. In a majority of such cases, heart disease is responsible for sudden death. Rare disease entities resulting in sudden death are occasionally encountered and may not fit the classic epidemiological profile. We present a case of sudden death due to a previously undiagnosed Wilms' tumor (WT) in an adult. The pathology of WT is discussed, as is the topic of sudden death due to previously unrecognized malignancy.
UI - 12065552
AU - Owens CM; Veys PA; Pritchard J; Levitt G; Imeson J; Dicks-Mireaux C
TI - Role of chest computed tomography at diagnosis in the management of Wilms' tumor: a study by the United Kingdom Children's Cancer Study Group.
SO - J Clin Oncol 2002 Jun 15;20(12):2768-73
AD - Department of Radiology, Great Ormond Street Hospital for Children National Health Service Trust, London, United Kingdom.
PURPOSE: This study sought to determine whether the identification of minimal pulmonary metastatic disease by chest computed tomography (CT) performed at diagnosis in patients with Wilms' tumor and normal chest x-rays (CXR) could predict a subgroup of children at increased risk of pulmonary relapse. PATIENTS AND METHODS: A retrospective analysis was carried out of the records of 449 children entered onto the United Kingdom Childrens' Cancer Study Group Second Wilms' Tumor Study between chest CT at diagnosis, but 141 children who had normal frontal and lateral CXRs and a chest CT scan performed at diagnosis were eligible for analysis. After surgery, children with stage I Wilms' tumor received single-agent chemotherapy (vincristine), whereas children with stages II, III, and bilateral Wilms' tumor received combination chemotherapy. Most children with stage III tumors were also treated with abdominal radiotherapy (20 Gy). RESULTS: In 31 patients (22%), pulmonary nodules were visible on chest CT; eight experienced relapse, four (15%) in the lungs. When only stage I patients were analyzed, there was a significant difference between the pulmonary relapse rate of 43% (three of seven) in the CT-positive group and 10% (five of 48) in the CT-negative group (P =.02). Four of eight patients with stage I disease with pulmonary relapse died. CONCLUSION: CT seemed to identify a subgroup of stage I patients who were at increased risk of pulmonary relapse. These children had received only single-agent chemotherapy. A prospective randomized trial is needed to clarify whether these children would benefit from combination chemotherapy.
UI - 12057921
AU - Li CM; Guo M; Borczuk A; Powell CA; Wei M; Thaker HM; Friedman R; Klein
TI - U; Tycko B Gene expression in Wilms' tumor mimics the earliest committed stage in the metanephric mesenchymal-epithelial transition.
SO - Am J Pathol 2002 Jun;160(6):2181-90
AD - Institute for Cancer Genetics, Columbia University, New York, New York, USA.
Wilms' tumor (WT) has been considered a prototype for arrested cellular differentiation in cancer, but previous studies have relied on selected markers. We have now performed an unbiased survey of gene expression in WTs using oligonucleotide microarrays. Statistical criteria identified 357 genes as differentially expressed between WTs and fetal kidneys. This set contained 124 matches to genes on a microarray used by Stuart and colleagues (Stuart RO, Bush KT, Nigam SK: Changes in global gene expression patterns during development and maturation of the rat kidney. Proc Natl Acad Sci USA 2001, 98:5649-5654) to establish genes with stage-specific expression in the developing rat kidney. Mapping between the two data sets showed that WTs systematically overexpressed genes corresponding to the earliest stage of metanephric development, and underexpressed genes corresponding to later stages. Automated clustering identified a smaller group of 27 genes that were highly expressed in WTs compared to fetal kidney and heterologous tumor and normal tissues. This signature set was enriched in genes encoding transcription factors. Four of these, PAX2, EYA1, HBF2, and HOXA11, are essential for cell survival and proliferation in early metanephric development, whereas others, including SIX1, MOX1, and SALL2, are predicted to act at this stage. SIX1 and SALL2 proteins were expressed in the condensing mesenchyme in normal human fetal kidneys, but were absent (SIX1) or reduced (SALL2) in cells at other developmental stages. These data imply that the blastema in WTs has progressed to the committed stage in the mesenchymal-epithelial transition, where it is partially arrested in differentiation. The WT-signature set also contained the Wnt receptor FZD7, the tumor antigen PRAME, the imprinted gene NNAT and the metastasis-associated transcription factor E1AF.
UI - 11842975
AU - Camassei FD; Ferlini C; Jenkner A; Bosman C; Biselli R; Donfrancesco A;
TI - Boldrini R Nephroblastoma. DNA characteristics and their modifications induced by prenephrectomy chemotherapy: a cytofluorimetric study.
SO - Pediatr Pathol Mol Med 2002 Jan-Feb;21(1):15-23
AD - Department of Pathology, Bambino Gesu Children's Hospital-Research Institute, Rome, Italy.
Treatment of nephroblastoma (Wilms' tumor) has presently achieved a 90% survival rate. Stage and grade are considered the most reliable prognostic parameters, but other biological factors are under study in order to improve patient stratification. Deoxyribonucleic acid (DNA) ploidy has been suggested to be useful in this setting. We retrospectively studied 79 patient with nephroblastoma (58 pretreated with chemotherapy and 21 not pretreated) by means of flow cytometry. DNA content and synthetic phase values were correlated with pathologic features and outcome. DNA modifications induced by chemotherapy were investigated. Sixty-nine tumors were diploid and 10 aneuploid. DNA content did not correlate with clinical course and was not modified by pretreatment. Aneuploid tumors were restricted to lower stages. Mean S-phase rate was lower and did not vary according to histology in pretreated tumors, while it was higher and increased with grade (p = 0.007) in previously untreated tumors. The fraction of cells in synthetic activity was related to outcome: Patients whose tumors displayed higher S-phase rates had a more favorable clinical course. Ploidy did not appear to be of prognostic significance. S-phase rate decreased after chemotherapy (p = 0.0002) and was related to survival. The worse outcome of pretreated patients might be attributed to a minor sensitivity to postoperative treatment: Preoperative chemotherapy would decrease the cell proliferation and might select resistant cellular clones of (possible) neoplastic residues.
UI - 11822711
AU - Amor DJ
TI - Morbid obesity and hyperphagia in the WAGR syndrome.
SO - Clin Dysmorphol 2002 Jan;11(1):73-4
A 33-year-old man with WAGR syndrome is described with morbid obesity associated with hyperphagia and an apparent lack of satiety. It is possible that a gene associated with satiety is present at 11p13 although it is premature to conclude that obesity is a specific feature of WAGR syndrome.
UI - 12085187
AU - White GR; Kelsey AM; Varley JM; Birch JM
TI - Somatic glypican 3 (GPC3) mutations in Wilms' tumour.
SO - Br J Cancer 2002 Jun 17;86(12):1920-2
AD - Cancer Research UK Cancer Genetics Group, Paterson Institute for Cancer Research, Wilmslow Road, Manchester M20 4BX, UK.
Tumour and normal tissue from 41 male cases of Wilms' tumour were screened to determine the presence of sequence variants in the glypican 3 (GPC3) gene. Two non-conservative single base changes were present in tumour tissue only. These findings imply a possible role for GPC3 in Wilms' tumour development. Copyright 2002 Cancer Research UK
UI - 12084281
AU - Ritchey ML
TI - Recent progress in the biology and treatment of Wilms' tumor.
SO - Curr Urol Rep 2001 Apr;2(2):127-31
AD - Division of Urology, University of Texas-Houston Medical, 6431 Fannin, 6.018, Houston, TX 77030, USA. email@example.com
The survival rate of patients with Wilms' tumor has rapidly improved in the last few decades. As we enter into the new millennium, overall survival for all Wilms' tumor patients exceeds 80%. Two large cooperative groups prospectively studied children with nephroblastoma: the National Wilms' Tumor Study Group, and the International Society of Paediatric Oncology. This review details the recent progress in understanding the biology of Wilms' tumor. Advances in clinical management of children with nephroblastoma are also outlined.
UI - 11837583
AU - Shiraishi A; Kuwatsuru R; Kurosaki Y; Maehara T; Fujita H; Miyano T;
TI - Matsumoto T Papillonodular type of cystic partially differentiated nephroblastoma: a case report.
SO - Radiat Med 2001 Nov-Dec;19(6):313-6
AD - Department of Radiology, Juntendo University School of Medicine, Tokyo, Japan.
We report a case of the papillonodular type of cystic partially differentiated nephroblastoma (CPDN), an extremely rare renal neoplasm that occurs in newborns and infants. The papillonodular type of CPDN is a variant of the conventional form of CPDN. MRI clearly demonstrated the gross pathologic features, distinguishing it from other renal multilocular cystic tumors.
UI - 11734691
AU - McLorie GA
TI - Wilms' tumor (nephroblastoma).
SO - Curr Opin Urol 2001 Nov;11(6):567-70
AD - Division of Urology, University of Toronto, and Hospital for Sick Children, Toronto, Ontario, Canada. firstname.lastname@example.org
Wilms' tumor (nephroblastoma) is a subject which continues to challenge clinicians in their attempts to achieve the best survival of their patients, while minimizing morbidity. Overall survival of over 85% of all children can now be achieved using combination therapy with chemotherapy, surgery, and in some cases radiotherapy. Recent reviews of two multi-institutional, multinational trials (the International Society of Pediatric Oncology and the National Wilms' Tumor Study Group) are discussed in this article, in which their current approaches to therapy are presented. In addition to these clinical advances, the genetic and molecular features of Wilms' tumor continue to provide insight into the biology of nephroblastoma and into neoplasia in general. A recent review of these aspects of the field is provided.
UI - 11882978
AU - Glekov IV; Lebedev VI; Belova VP; Dzhabarov FR; Danilova VS; Boichenko
TI - EI; Sharoev TA [Radiotherapy in multiple modality treatment of children with nephroblastoma]
SO - Vestn Ross Akad Med Nauk 2002;(1):68-71
The role of radiotherapy in multiple modality treatment of Wilms' tumor is evaluated in 225 children aged 3 months to 11.5 years (mean age 3.5 years) with stage III-IV. 184 (81.8%) patients presented with stage III, 93.7% with typical nephroblastoma. Intervention was combined with drug and radiotherapy in 99.6% patients. Exposure of the abdominal cavity in total focal doses of 10.5-50.2 Gy (mean dose 28 Gy) was carried out in 219 (97.3%) of 225 patients. Special attention is paid to the incidence of relapses, remote metastases, and survival of patients in relation to prognostic factors (sex, age, stage of tumor process, terms of exposure, and total focal doses). All patients were followed up for 2-203 months (median 32 months). During this period relapses and/or metastases were observed in 34.2% patients; 30.2% died because of disease progress. 35.1% children live without signs of disease for more than 5 years, 14.7% for more than 10 years. Relapses were more incident during the first year of treatment (in 65% children) and outside the exposed field (72.5% cases). 33.2% patients with stage III developed metastases after 1-49 months; the lungs were involved most often. Prolongation of the period between surgery and exposure of the abdominal cavity led to increase in the incidence of relapses in the abdominal cavity from 6.7% (up to 2 weeks) to 21.9% (more than 1 month), p = 0.02. Relapses were the most frequent in children aged over 4 years. This parameter virtually did not depend on the total focal dose. The absence of relationship between the incidence of local relapses and life span after exposure to a total focal dose of up to 21.6 Gy in comparison with higher doses recommends reduced doses for therapy without notable deterioration of the survival of patients with nephroblastoma.
UI - 11803784
AU - Carmona Campos E; Zarate Rodriguez E
TI - [Wilms tumor in adults. Report of a case]
SO - Actas Urol Esp 2001 Nov-Dec;25(10):755-8
AD - Unidad de Urologia, Hospital Alto Guadalquivir, Andujar, Jaen.
OBJECTIVE: Wilms' tumor or nephroblastoma is the most common renal tumor of childhood. The incidence in adults is rare, estimated at about 1% of all cases and approximately 200 cases have been described in the world literature. We report a new case of adult Wilms' tumor and realize a short review. METHODS/RESULTS: We describe a case of Wilms' tumor in a young male, aged 15 years, with associate genitourinary abnormalities. The patient underwent radical nepherctomy with postoperative chemotherapy. CONCLUSIONS: The Wilms' tumor in the adult population is exceptional, and the definitive diagnostic is based in the findings of the pathological analysis. The application of therapeutic protocolos for children to adults offers an smaller percentage of cure and poorer prognostic. Therapheutic protocolos more aggressive are necessary for increase the worse prognostic in the adult, but because of the rarity is difficult to define the most effective form of treatment.
UI - 12116068
AU - Muwakkit S; Antillon F; Valverde P; Jenkins JJ 3rd; Zaatari G; Dome JS
TI - Simultaneous occurrence of Wilms tumor and rhabdomyosarcoma in two patients.
SO - Med Pediatr Oncol 2002 Aug;39(2):143-5
UI - 12113088
AU - Ehrlich PF
TI - Wilms tumor: progress to date and future considerations.
SO - Expert Rev Anticancer Ther 2001 Dec;1(4):555-64
AD - West Virginia University School of Medicine, Morgantown, WV, USA. email@example.com
Wilms tumor is the most common tumor of renal origin found in children. In the last 50 years, remarkable progress has been made in the treatment and understanding of children with Wilms tumor. Through the development of multiagent chemotherapy and cooperative pediatric interdisciplinary groups conducting large randomized controlled clinical trials, survival has improved dramatically. In the next century it is expected that 80% of children with Wilms tumor will be long-term survivors. Therapy is progressing towards a risk-based management based not only on stage and histology but also incorporated genetic markers. This article reviews progress to date and possible future directions in the treatment of Wilms Tumor.
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