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Cancer Types / Pediatric Cancers / Wilms' Tumor / NCI Resources

NCI CANCERLIT^® Search: Wilms' Tumor - October 2002

National Cancer Institute^®
Last Modified: October 1, 2002

Chromosome arm 16q in Wilms tumors: unbalanced chromosomal translocations, loss of heterozygosity, and assessment of the CTCF gene.
[Enormous tumour in the right atrium of a Wilms' tumour patient]
Hypomethylation and hypermethylation of DNA in Wilms tumors.
Hemolytic uremic syndrome associated with Denys-Drash syndrome.
Controversies and advances in the management of Wilms' tumour.
A developmental study of novH gene expression in human central nervous system.
Treated Wilm's tumor in childhood as potential risk factor for second thyroid cancer.

Table of Contents

1
UI - 12203779
AU - Yeh A; Wei M; Golub SB; Yamashiro DJ; Murty VV; Tycko B
TI - Chromosome arm 16q in Wilms tumors: unbalanced chromosomal translocations, loss of heterozygosity, and assessment of the CTCF gene.
SO - Genes Chromosomes Cancer 2002 Oct;35(2):156-63
AD - Department of Pathology, Columbia University College of Physicians and Surgeons, New York, New York, USA.
Chromosome arm 16q is a common site of loss of heterozygosity (LOH) in Wilms tumors (WTs). The mechanism and consequences of 16q LOH are not known, but the CTCF gene, in band 16q22, is a candidate target gene. CTCF protein binds to DNA upstream of the H19 gene on chromosome band 11p15, and maintains normal imprinting of H19 and IGF2. Thus, its loss might predispose to de novo methylation of the maternal allele of H19 and loss of imprinting (LOI) of IGF2 in WTs. We mapped Chr16 LOH in WTs and correlated this with unbalanced chromosomal translocations and histopathology. We also analyzed the CTCF gene for its mRNA and protein expression and DNA sequence, and we investigated correlations with methylation of H19(mat). In our series, unbalanced t(1;16) chromosomal translocations were a major pathway for Chr16 loss of heterozygosity, and this LOH was correlated significantly with tumor anaplasia. CTCF mapped in the minimal region of Chr16 LOH. However, we found no correlation between Chr16 LOH, or loss of CTCF mRNA or protein, and methylation of H19(mat). Some WTs contained reduced amounts of CTCF protein, but among these were cases with and without H19(mat) methylation. Rare WTs with simultaneous 16q LOH and H19(mat) methylation lacked CTCF mutations. These data argue against the CTCF gene as a target of Chr16 LOH in WTs, but leave open the possibility that post-transcriptional loss of CTCF protein may account for some instances of LOI. Copyright 2002 Wiley-Liss, Inc.

2
UI - 11093345
AU - Aleszewicz-Baranowska J; Stefanowicz J; Balcerska A
TI - [Enormous tumour in the right atrium of a Wilms' tumour patient]
SO - Med Wieku Rozwoj 2000 Jul-Sep;4(3):277-83
AD - Klinika Kardiologii Dzieciecej Instytutu Pediatrii, Akademia Medyczna, Debinki 7, 80-211, Gdansk, Poland.
Formation of neoplastic thrombus in vena cava inferior (VCI) is well known in Wilms' tumour. We demonstrate a case of large neoplastic mass in the right atrium as prolongation from vena cava inferior in a 2 years and 2 months old girl with Wilms' tumour. The long preoperative chemotherapy (7 months) reduced the tumour and the thrombus. The surgical removal of the tumour and the thrombus was followed by further chemotherapy. During postoperative chemotherapy the control USG examinations showed the presence of a stable mass in a caval wall (a neoplastic mass or a scar?). The extension of the thrombus into the right atrium remains a therapeutic problem which has not been yet resolved.

3
UI - 12242669
AU - Ehrlich M; Jiang G; Fiala E; Dome JS; Yu MC; Long TI; Youn B; Sohn OS;
TI - Widschwendter M; Tomlinson GE; Chintagumpala M; Champagne M; Parham D; Liang G; Malik K; Laird PW Hypomethylation and hypermethylation of DNA in Wilms tumors.
SO - Oncogene 2002 Sep 26;21(43):6694-702
AD - Tulane Cancer Center and Human Genetics Program, Tulane Medical School, New Orleans, Louisiana, LA 70112, USA. ehrlich@tulane.edu
We quantitatively analysed hypermethylation at CpG islands in the 5' ends of 12 genes and one non-CpG island 5' region (MTHFR) in 31 Wilms tumors. We also determined their global genomic 5-methylcytosine content. Compared with various normal postnatal tissues, approximately 40-90% of these pediatric kidney cancers were hypermethylated in four of the genes, MCJ, RASSF1A, TNFRSF12 and CALCA as determined by a quantitative bisulfite-based assay (MethyLight). Interestingly, the non-CpG island 5' region of MTHFR was less methylated in most tumors relative to the normal tissues. By chromatographic analysis of DNA digested to deoxynucleosides, about 60% of the Wilms tumors were found to be deficient in their overall levels of DNA methylation. We also analysed expression of the three known functional DNA methyltransferase genes. No relationship was observed between global genomic 5-methylcytosine levels and relative amounts of RNA for DNA methyltransferases DNMT1, DNMT3A, and DNMT3B. Importantly, no association was seen between CpG island hypermethylation and global DNA hypomethylation in these cancers. Therefore, the overall genomic hypomethylation frequently observed in cancers is probably not just a response or a prelude to hypermethylation elsewhere in the genome. This suggests that the DNA hypomethylation contributes independently to oncogenesis or tumor progression.

4
UI - 11045393
AU - Sherbotie JR; van Heyningen V; Axton R; Williamson K; Finn LS; Kaplan BS
TI - Hemolytic uremic syndrome associated with Denys-Drash syndrome.
SO - Pediatr Nephrol 2000 Oct;14(12):1092-7
AD - Department of Pediatrics, The Children's Hospital of Philadelphia and University of Pennsylvania, 19104, USA.
The Denys-Drash syndrome is defined by the occurrence of combinations of pseudohermaphroditism, nephrotic syndrome with diffuse mesangial sclerosis, Wilms' tumor, and constitutional mutations in the WT1 suppressor gene. Most patients develop end-stage renal failure. Atypical hemolytic uremic syndrome (HUS) is defined by onset of acute hemolytic anemia with fragmented erythrocytes, thrombocytopenia, and renal failure in the absence of a gastrointestinal prodromal illness of bloody diarrhea. The purpose of this report is to describe the occurrence of features of atypical HUS and Denys-Drash syndrome in two African-American boys aged 13 and 16 months. Each had nephrotic syndrome, diffuse mesangial sclerosis, and WT1 point mutations. Both had grade III hypospadias and undescended testes. They had normal serum creatinine concentrations and hematology a month before presenting with HUS. Stool cultures for Escherichia coli O157:H7 were negative. Each patient has been transplanted with cadaver kidneys without recurrence of HUS.

5
UI - 12193442
AU - Pritchard-Jones K
TI - Controversies and advances in the management of Wilms' tumour.
SO - Arch Dis Child 2002 Sep;87(3):241-4
AD - The Institute of Cancer Research/Royal Marsden Hospital, Section of Paediatric Oncology, Sutton, Surrey, UK. kpj@icr.ac.uk
Wilms tumour is one of the success stories of paediatric oncology with long term survival approaching 90% in localised disease and over 70% for metastatic disease. Although appearing relatively simple compared to other cancer treatment regimens, successful treatment of Wilms tumour requires meticulous attention to correct staging of the tumour and good communication between the paediatric surgeon, pathologist and oncologist. The controversy of whether pre-operative chemotherapy results in a reduced overall burden of treatment compared to immediate nephrectomy has been addressed by the recently closed UKW3 randomised trial. Challenges remain in identification of histological and molecular risk factors for stratification of treatment intensity to allow safe reduction in therapy and avoidance of late sequelae for the majority while leading to increased biological insights and ultimately novel therapies for the minority of high risk tumours. Genetic predisposition to Wilms tumour is conferred by several genes, some of which cause malformation rather than cancer and may be of low penetrance. The proportion of children with heritable disease is uncertain and there remains a need to collect data on the need for screening in this susceptible population.

6
UI - 9879467
AU - Su BY; Cai WQ; Zhang CG; Su HC; Perbal B
TI - A developmental study of novH gene expression in human central nervous system.
SO - C R Acad Sci III 1998 Nov;321(11):883-92
AD - Department of Histology and Embryology, Third Military University, Chongqing, P.R. China.
The expression pattern of the human nephroblastoma overexpressed (novH) gene in the fetal human central nervous system was examined by in situ hybridization using digoxigenin-labeled novH-specific riboprobes. In the spinal cord, the nov-expressing neurons were first detected both in the ventral region at 16 weeks of gestation (G16W) and in the dorsal region at G38W. In the medulla, nov-expressing neurons were detected in the principal nucleus of the inferior olive, the hypoglossal nucleus and the dorsal motor nucleus of vagus at G16W. Nov-positive neurons were detected at G28W in the nucleus of the spinal tract of the trigeminal and cuneate nucleus, and at G38W in the abducens nucleus of pons, the red nucleus and the substantia nigra of the midbrain, the ventral posterolateral and the mediodorsal thalamic nucleus. A strong labeling was also detected in the striatum of the cerebrum and the cerebral cortex of the parietal lobe. These data established that novH is mainly expressed in somato-motor neurons in the lower central nervous system at early developmental stages and in the higher central nervous system at later stages, suggesting that nov may play an important role in neuronal differentiation.

7
UI - 12094146
AU - Vezzadini C; Cremonini N; Sforza A; Presutti L; Chiarini V
TI - Treated Wilm's tumor in childhood as potential risk factor for second thyroid cancer.
SO - Panminerva Med 2002 Sep;44(3):275-7
AD - Operative Unit of Endocrinology and Metabolic Diseases, Bellaria and Maggiore Hospital, Bologna, Italy.
The potential risk of a treatment-induced second neoplasia affecting the thyroid is well known after radiation therapy for several types of cancer, but few cases have been related to incidental irradiation for Wilms' tumor. We report a case of a papillary thyroid carcinoma discovered in a young patient 15 years after treatment of a Wilms' tumor. An 18-year-old man was referred to our Endocrinological Department for a single 3 cm nodule in the right lobe of the thyroid. His past medical history included at the age of 2 years surgical resection, chemotherapy (actinomycin-D and vincristine) and cesium radiation therapy to the right side for a Wilms' tumor in stage III: a total dose of 7700 rads was delivered to an area of 17 x 10 cm in the right flank. After fine-needle demonstration of a follicular thyroid lesion, the patient underwent right lobectomy, followed by total thyroidectomy for histologic diagnosis of a follicular variant papillary cancer. Residual thyroid tissue was ablated by iodine-131 administration (3700 MBq), but scanning after therapeutic iodine showed radioactive uptake in the left regional lymph nodes, with elevated serum thyroglobulin off therapy (830 ng/ml). Magnetic resonance imaging confirmed the presence of lymph node enlargements and bilateral neck dissection was performed, followed by radioiodine treatment (3700 MBq) and thyroxine suppressive therapy. After 3-year follow-up the patient is disease-free. Although few cases of thyroid cancer have been reported in the literature after irradiation for a Wilms' tumor during childhood, this association should be considered in the long-term follow-up.

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