National Cancer Institute®
Last Modified: November 21, 2001
UI - 21338548
AU - Efferth T; Thelen P; Schulten HG; Bode ME; Granzen B; Beniers AJ;
TI - Mertens R; Ringert RH; Gefeller O; Jakse G; Fuzesi L Differential expression of the multidrug resistance-related protein MRP1 in the histological compartments of nephroblastomas.
SO - Int J Oncol 2001 Aug;19(2):367-71
AD - Virtual Campus Rhineland-Palatinate, P.O. Box 4380, G-55033 Mainz, Germany. email@example.com
Nephroblastomas (Wilms' tumors) are curable with survival rates above 80%. Some tumors, however, fail to respond to therapy and those patients have a poor prognosis. In a search for prognostic markers, we investigated the expression of the multidrug resistance-related protein 1 (MRP1) in 32 nephroblastomas by means of immunohistochemistry. The immunohistochemical results were validated with a real-time RT-PCR technique. MRP1 expression was heterogeneous and predominantly found in the blastemal and epithelial compartments compared to the stromal elements of nephroblastomas. We found significant relationships of MRP1 expression to survival of patients and to expression of p53, HSP70, and LRP/MVP. The relationship between MRP1 and p53 expression is a clue that the transcriptional control of MRP1 by p53 reported for other tumor types may also take place in nephroblastomas. The correlation of MRP1 to other drug resistance genes, e.g. HSP70 and LRP/MVP in nephroblastomas indicates that the co-expression of different drug resistance genes may be under a common regulation of still unknown transcription factors.
UI - 21450467
AU - Fernandez CV; Lestou VS; Wildish J; Lee CL; Sorensen PH
TI - Detection of a novel t(6;15)(q21;q21) in a pediatric Wilms tumor.
SO - Cancer Genet Cytogenet 2001 Sep;129(2):165-7
AD - Department of Pediatrics, Dalhousie University, Halifax, Nova Scotia, Canada. firstname.lastname@example.org
We report a novel cytogenetic finding in a favorable histology Wilms tumor occurring in a 4-month-old boy. Karyotypic analysis demonstrated a t(6;15)(q21;q21) in all tumor cells examined. This was confirmed using fluorescence in situ hybridization analysis. Molecular analysis of this rearrangement may provide clues to understanding the pathobiology of Wilms tumor.
UI - 21263121
AU - Godzinski J; Weirich A; Tournade MF; Gauthier F; Buerger D;
TI - Moorman-Voestermans CG; de Kraker J; Voute P; Ludwig R; Sawicz-Birkowska K; Vujanic G; Ducourtieux M Primary nephrectomy for emergency: a rare event in the International Society of Paediatric Oncology Nephroblastoma Trial and Study no. 9.
SO - Eur J Pediatr Surg 2001 Feb;11(1):36-9
AD - Department of Paediatric Surgery, Marciniak Hospital, Wroclaw, Poland. email@example.com
Experience of the International Society of Paediatric Oncology (SIOP) Trials and Studies indicates that the preoperative chemotherapy in Wilms' tumour improves stage distribution, decreases complication rate and reduces postoperative treatment. However, some situations may lead to prompt primary surgery. The aim of the study is to assess reasons leading to primary emergency nephrectomy. Records of 720 patients with non-metastatic unilateral nephroblastoma who were registered in the SIOP Trial and Study 9 were reviewed. Twenty-four (3%) cases of primary emergency nephrectomy were identified. Reasons leading to emergency nephrectomy were massive bleedings from ruptured tumours in 13 patients, suspicion of an "acute abdomen" in 7, bowel occlusion in 2 and other in 2. Postoperative treatment included radiotherapy in 71% of cases and anthracyclines in 92%. Complications were frequent and happened in 25% of patients, the outcome however, was favourable and 22 of 24 patients are alive (from 9 to 79 months). The 7 patients with a suspicion of an "acute abdomen" probably constitute the group which could have been markedly reduced if adequately diagnosed and observed prior to surgery.
UI - 21328976
AU - Gaston V; Le Bouc Y; Soupre V; Burglen L; Donadieu J; Oro H; Audry G;
TI - Vazquez MP; Gicquel C Analysis of the methylation status of the KCNQ1OT and H19 genes in leukocyte DNA for the diagnosis and prognosis of Beckwith-Wiedemann syndrome.
SO - Eur J Hum Genet 2001 Jun;9(6):409-18
AD - Laboratoire d'Explorations Fonctionnelles Endocriniennes, Hopital Trousseau, AP-HP, Paris, France.
Beckwith-Wiedemann syndrome (BWS) is an overgrowth disorder involving developmental abnormalities, tissue and organ hyperplasia and an increased risk of embryonal tumours (most commonly Wilms tumour). This multigenic disorder is caused by dysregulation of the expression of imprinted genes in the 11p15 chromosomal region. Molecular diagnosis of BWS is currently difficult, mostly due to the large spectrum of genetic and epigenetic abnormalities. The other difficulty in managing BWS is the identification of patients at risk of tumour. An imprinted antisense transcript within KCNQ1, called KCNQ1OT (also known as LIT1), was recently shown to be normally expressed from the paternal allele. A loss of imprinting of the KCNQ1OT gene, associated with the loss of maternal allele-specific methylation of the differentially methylated region KvDMR1 has been described in BWS patients. The principal aim of this study was to evaluate the usefulness of KvDMR1 methylation analysis of leukocyte DNA for the diagnosis of BWS. The allelic status of the 11p15 region and the methylation status of the KCNQ1OT and H19 genes were investigated in leukocyte DNA from 97 patients referred for BWS and classified into two groups according to clinical data: complete BWS (CBWS) (n=61) and incomplete BWS (IBWS) (n=36). Fifty-eight (60%) patients (39/61 CBWS and 19/36 IBWS) displayed abnormal demethylation of KvDMR1. In 11 of the 56 informative cases, demethylation of KvDMR1 was related to 11p15 uniparental disomy (UPD) (nine CBWS and two IBWS). Thirteen of the 39 patients with normal methylation of KvDMR1 displayed hypermethylation of the H19 gene. These 13 patients included two siblings with 11p15 trisomy. These results show that analysis of the methylation status of KvDMR1 and the H19 gene in leukocyte DNA is useful in the diagnosis of 11p15-related overgrowth syndromes, resulting in the diagnosis of BWS in more than 70% of investigated patients. We also evaluated clinical and molecular features as prognostic factors for tumour and showed that mosaicism for 11p15 UPD and hypermethylation of the H19 gene in blood cells were associated with an increased risk of tumour.
UI - 21372318
AU - Thomson GD; Blair GK
TI - Multislice helical CT depiction of Wilms' Tumor.
SO - J Pediatr Surg 2001 Aug;36(8):1313-4
AD - Department of Radiology, British Columbia's Children's Hospital, Vancouver BC, Canada.
UI - 21263173
AU - Wunsch L; Flemming P; Gluer S
TI - Expression of MIB and BCL-2 in patients with nephrogenic rests with and without associated Wilms' tumors.
SO - Eur J Pediatr Surg 2001 Apr;11(2):105-9
AD - Department of Pediatric Surgery, Hannover Medical School, Germany. firstname.lastname@example.org
Nephrogenic rests (NR) are foci of persistent embryonal renal tissue. Because it has been suggested that NRs are precursor lesions to Wilms' tumor (WT), they are of considerable clinical interest. NRs vary from microscopic foci to macroscopic renal tumors, but only a few progress to WT. In this study, patients with NRs detected during the treatment of bilateral WT were compared to a group of patients with NRs incidentally discovered in various clinical settings. Because mechanisms leading to NR growth and WT formation are poorly understood, bcl-2 and MIB expression were studied by immunohistochemistry in both groups. Bcl-2 is an oncoprotein with inhibitory effects on apoptosis and MIB is a well-established marker of cell proliferation. Both mechanisms may be of interest for the growth, regression and transformation of NRs. Intense positive staining for bcl-2 was found in microscopic NRs. Blastemal cells and cells with epithelial differentiation were bcl-2-positive. The same pattern of bcl-2 expression was found in NRs with and without associated WT. High proliferative activity with intense MIB expression was found in blastemal areas of WT. Bcl-2 expression in NR is reported for the first time. Inhibition of apoptosis as a mechanism of NR formation is suggested. This is of special interest, because bcl-2 is under transcriptional control of the WT-1 gene.
UI - 21263171
AU - Trobs RB; Hansel M; Friedrich T; Bennek J
TI - A 23-year experience with malignant renal tumors in infancy and childhood.
SO - Eur J Pediatr Surg 2001 Apr;11(2):92-8
AD - Klinik und Poliklinik fur Kinderchirurgie, Universitat Leipzig, Germany.
A retrospective analysis of 77 children treated between 1974 and 1996 was undertaken to evaluate morbidity and the evolution of therapy. A Wilms' tumor (WT) was present in 73 children. 74% of patients (pats.) with WT survived (54 of 73 pats.). Histological specimens of 67 patients were re-evaluated, including 4 children with non-WT histology. Among patients with Wilms' tumors (WT), nephroblastoma (NB) of intermediate risk predominated (73%; 46 of 63 pats.). Low-risk tumors occurred in 5 of 63 children (8%; mesoblastic nephroma 3, cystic partially diff. NB 1, completely necrotic NB 1). High-risk WT were diagnosed in 12 of 63 patients (19%) (NB with anaplasia 10, clear cell sarcoma 1, malignant rhabdoid tumor 1). Nephrogenic rests were present in 14 cases. We observed 3 children of school age with renal carcinoma and one patient with an intrarenal neuroblastoma. WT histology was the most important factor determining prognosis (p = 0.018). The risk for relapses was 2.6-fold higher in patients with high-risk WT compared to the standard risk group. Stages were re-evaluated according to SIOP 93-01. Comparing relapse-free survival of stages I, II and III, respectively, there was a reduced survival rate for stage III (p=0.019). According to the SIOP/GPOH protocol in 1989, the regimen was switched from primary surgery to preoperative chemotherapy without biopsy in 1989 (11 pats.). Compared to earlier years, survival improved (n.s.). In 3 patients preoperative diagnosis by means of imaging failed. During preoperative chemotherapy a venous occlusive disease of the liver occurred in 2 patients. Preoperative chemotherapy led to an impressive tumor shrinkage in the majority of patients. 2 patients of the preoperative group died (focal anaplastic NB). Long-term morbidity was analysed in 49 patients and included radiation-induced scoliosis (35), chest-wall deformity (3), congestive cardiomyopathy after relapse (1) and arterial hypertension (2). Over the years there was a trend to reduce frequency and dose of irradiation. Prognosis of WT is excellent but unfavorable histology (high risk) predicts a poor prognosis. In our experience, reduction of tumor volume due to preoperative chemotherapy facilitates tumor removal by surgery and may prevent tumor spillage and the deleterious effects of radiation in young children. Surgery without delay is necessary if the diagnosis is unclear or the tumor fails to respond to preoperative chemotherapy.
UI - 21263172
AU - Paya K; Horcher E; Lawrenz K; Rebhandl W; Zoubek A
TI - Bilateral Wilms' tumor--surgical aspects.
SO - Eur J Pediatr Surg 2001 Apr;11(2):99-104
AD - Department of Pediatric Surgery, University of Vienna, Austria. email@example.com
Four to eight percent of all Wilms' tumors are bilateral. Achieving curative resection of such tumors by partial nephrectomy or tumor enucleation while maintaining sufficient renal function represents a surgical challenge. Effective preoperative chemotherapy facilitates this aim considerably. Seven patients who were diagnosed to have bilateral synchronous Wilms' tumors between 1990 and 1994 are being reviewed. At the time of initial diagnosis, their mean age was 24.4 months, range 7 to 45 months. In 4 cases, pre-operative imaging did not reveal the full extent of the lesions, and in one of these, involvement of the second kidney was only detected by surgical exploration. Five of the children received at least 4 weeks of neo-adjuvant chemotherapy without primary biopsy, followed by bilateral tumor resection. One child had to be operated on as an emergency without any preliminary treatment, and in one, chemotherapy was interrupted after 3 weeks because of veno-occlusive disease. After a follow-up period of 66 months on the average (range 50 to 81 months), five of the patients are free of recurrence and clinically well--one with a kidney graft. The remaining two patients have died. Discussion is focussed on different management strategies of this rather rare pathology considering SIOP und NWTS protocols.
UI - 21296217
AU - Roberg K
TI - Kelsey's story.
SO - Am J Health Syst Pharm 2001 Jun 1;58(11):985-7
AD - Delphi Ventures, 3000 Sand Hill Road, Building 1, Suite 135, Menlo Park, CA 94025, USA.
UI - 21314242
AU - Schuz J; Kaletsch U; Meinert R; Kaatsch P; Michaelis J
TI - High-birth weight and other risk factors for Wilms tumour: results of a population-based case-control study.
SO - Eur J Pediatr 2001 Jun;160(6):333-8
AD - Institut fur Medizinische Statistik und Dokumentation, Johannes Gutenberg-Universitat Mainz, Germany. firstname.lastname@example.org
Wilms tumour, or nephroblastoma, is one of the childhood cancers included in two recent population-based case-control studies in West Germany. Altogether, 177 children under the age of 10 years with Wilms tumour diagnosed between 1988 and 1994 and 2006 control children sampled from population registration files participated. Information on potential risk factors was obtained from the parents using a questionnaire and by subsequent telephone interview. We found an association with a high birth weight >4000 g (odds ratio 1.58; 95% confidence interval 1.01-2.48), which was somewhat stronger for children aged 2 years or older. Findings for young maternal age at birth and certain parental occupationally related exposures were not reported by previous studies and thus may be chance findings. As opposed to previous studies, we failed to confirm associations with high parental age at birth, maternal coffee and tea consumption during pregnancy, and exposure to pesticides. CONCLUSION: Based on this large population-based case-control study, high birth weight may play a role in the aetiology of Wilms tumour, but many risk factors previously suggested are of less importance.
UI - 21461222
AU - Guler E; Emir S; Kutluk T; Varan A; Buyukpamukcu M
TI - Urticaria pigmentosa associated with Wilms tumor.
SO - Pediatr Dermatol 2001 Jul-Aug;18(4):313-5
AD - Department of Pediatric Oncology, Hacettepe University Institute of Oncology, Ankara, Turkey.
Urticaria pigmentosa is the most common manifestation of mastocytosis, with the majority of cases undergoing spontaneous resolution, especially in children. Several reports have documented hematologic malignancies developing in patients with urticaria pigmentosa. We present a 4.5-year-old boy with urticaria pigmentosa who developed Wilms tumor. To our knowledge, coexisting urticaria pigmentosa and Wilms tumor have not previously been described.
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