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At least 19 recordsLinked to original sources
PubMed1995

[Hereditary renal tumors: Wilms' tumor--congenital anomalies' syndrome].

The genetics and associated abnormalities of Wilms' tumor are reviewed. Wilms' tumor is associated with several congenital syndromes such as WAGR (Wilms' tumor, aniridia, genitourinary malformation, mental retardation) syndrome, Denys-Drash syndrome, Beckwith-Wiedemann syndrome, etc. However, the association with such syndromes is relatively infrequent and accounts for less than 5% of all clinical patients with Wilms' tumor. WAGR syndrome and Denys-Drash syndrome are associated with loss of WT1 gene located in the chromosome 11p13, and BW syndrome is considered to be due to duplication of the paternal 11p15 allele (WT2). The association of Wilms' tumor with primary brain tumors in a daughter and a mother is also described.

Age of Onset↗
PubMed1981

Retroperitoneal malignant tumors: Wilms' tumor and neuroblastoma.

Wilms' tumor is one of the most curable of all childhood cancers, with a five-year survival of approximately 80 per cent. Survival of children with neuroblastoma, on the other hand, is dismal. For both tumors the primary mode of treatment is surgical excision, which should be carried out as an integrated part of multidisciplinary treatment.

Child↗
PubMed1993

Management of synchronous bilateral Wilms tumor: Brazilian Wilms Tumor Study Group experience with 14 cases. Brazilian Wilms Tumor Cooperative Group, Sao Paulo, Brazil.

The Brazilian Wilms Tumor Study Group has registered 16 cases of synchronous bilateral Wilms tumor since 1986, of which 14 were available for analysis. Most patients were treated according to a prospective trial in which bilateral renal preservation was emphasized. Overall survival was 79% (11 of 14 cases), and both kidneys were preserved in 57% (8 of 14) of the patients with synchronous disease. Of the 14 patients 5 had undergone at least 3 surgical procedures. In 10 cases in which pathological reports were available for review residual disease was documented in 5 but none had relapse.

Child↗
PubMed1984

High-resolution studies in patients with aniridia-Wilms tumor association, Wilms tumor or related congenital abnormalities.

We attempted to determine whether all cases of AWTA (anirida-Wilms tumor association) or any of the following groups of patients show 11p deletion: cases of Wilms tumor with congenital abnormalities other than aniridia, those without any congenital abnormalities, tumor itself in cases of Wilms tumor without constitutional 11p deletion and cases of aniridia or hemihypertrophy without Wilms tumor. We studied a total of 29 index patients including five cases of AWTA, four cases of Wilms tumor with various congenital abnormalities, 16 cases of Wilms tumor without other abnormalities, three cases of aniridia in one of which Wilms tumor developed later and a case of hemihypertrophy. In all five cases of AWTA and in a case of aniridia who later developed Wilms tumor, 11p deletion involving the p13 band was detected. The mother of the latter also showed an identical 11p deletion. The common segment of deletion was the middle part of the p13. Two possible hypotheses on the mechanism through which Wilms tumor might develop were evaluated, based on the distribution of break points. All other cases, including five with tumor culture, showed a normal karyotype.

Adolescent↗
PubMed1982

Metachronous bilateral Wilms' tumor, National Wilms' Tumor Study.

Eighteen of the patients randomized for treatment according to the first and second National Wilms' Tumor Studies have developed clinically evident tumor in the contralateral kidney subsequent to the initial diagnosis. Only seven of 18 had an adequate exploration of the opposite kidney during the initial surgery and of these seven, four had abnormal findings. Thirteen of 18 cases showed some form of nephroblastomatosis histologically. The original tumors in six and possibly seven were multicentric in origin. While metachronous Wilms' tumors are an unusual occurrence, the prognosis is ominous with only seven of 18 surviving free of disease. The outlook might be improved with adequate attention to the surgical and histologic details during the initial diagnostic procedures.

Child, Preschool↗
PubMed1998

Long-term renal function in patients with irradiated bilateral Wilms tumor. National Wilms' Tumor Study Group.

The treatment of bilateral Wilms tumor (BWT) involves a multidisciplinary approach including surgery, chemotherapy, and radiation therapy. The long-term renal function in patients receiving all three treatment modalities has not been evaluated. Long-term renal function was evaluated in 81 children with synchronous BWT who received radiation therapy as part of their treatment. Renal function was assessed by measuring blood urea nitrogen (BUN) and serum creatinine (Cr). The normal range for the BUN was defined as 10-24 mg/dl, and the Cr was considered normal at levels of <1.5 mg/dl. Moderate elevations were defined as a BUN of 25-50 mg/dl and/or a Cr of 1.6-2.5 mg/dl and marked elevations as a BUN of >50 mg/dl and/or a Cr of >2.5 mg/dl. BUN and Cr levels were measured prior to treatment and at the following intervals: 6 months after treatment, 1 year after treatment, 2 years after treatment, and at last follow-up. Any elevation during the posttreatment follow-up period was considered abnormal. A total of 28 children (34.6%) had elevated BUN and/or Cr levels, and 18 had moderate and 10 had marked renal insufficiency. No dose-response relationship was established when comparing the radiation doses of those with elevated values to those with normal values. The renal complication rate was moderate, and other factors including surgery, extent and nature of chemotherapy, and recurrent tumor must also be taken into account. The elevations present in several children could be attributed to tumor recurrence and in one case to gentamicin toxicity. The management of children with BWT should consider all of these risks, and attempts to preserve renal parenchyma are warranted.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1996

Use of dipyrone during pregnancy and risk of Wilms' tumor. Brazilian Wilms' Tumor Study Group.

We evaluated the risk of Wilms' tumor in the offspring of women taking various medications during pregnancy in a case, control study conducted in Brazil. The study accrued 109 cases and 218 age- and gender-matched hospital controls. After adjustment for known confounders, we found a strong association with ingestion of dipyrone (odds ratio = 10.9; 95% confidence interval = 2.4-50) particularly in women from low-income families. Although dipyrone-containing analgesics are banned in Europe and North America, they are widely prescribed in Brazil and are given as free samples in neighborhood clinics providing free health care. The strong effect specific to low-income women may result from higher individual consumption compared with women at higher income levels.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1993

Homozygous somatic Wt1 point mutations in sporadic unilateral Wilms tumor.

Wilms tumor may be caused by loss of function of genes at different loci. A Wilms tumor suppressor gene, WT1, at chromosome 11 band p13, has recently been cloned and characterized. WT1 has been implicated in the development of Wilms tumor by virtue of mutations in patients with genitourinary anomalies and susceptibility to Wilms tumor. Homozygous intragenic mutations have been reported in Wilms tumors, but usually not in sporadic unilateral Wilms tumors, which constitute the majority of Wilms tumor cases. Using the single-strand conformational polymorphism assay, we have identified three sporadic unilateral Wilms tumors with homozygous point mutations: one with a de novo germ-line nonsense point mutation within WT1 exon 8, and two carrying a somatic mutation within WT1 exon 10. In all three cases loss of the wild-type allele was demonstrated by tumor loss of heterozygosity. This report provides an example of two somatic mutations in the same tumor expected to inactivate WT1 function.

Amino Acid Sequence↗
PubMed1997

Aberrant imprinting of the insulin-like growth factor II receptor gene in Wilms' tumor.

Wilms' tumor (WT) is an embryonal renal malignancy, which overexpresses insulin-like growth factor II (IGF-II), a fetal mitogen. Relaxation of parental imprinting of IGF2, the gene encoding IGF-II, is found in Wilms' tumors, suggesting an important role for IGF2 dosage in tumorigenesis. The IGF2R gene encodes a nonmitogenic receptor which targets IGF-II to the lysosomes for degradation and, therefore, inhibits the mitogenic function of IGF-II. The human IGF2R is imprinted in a proportion of normal individuals. To test the hypothesis that IGF2R imprinting predisposes to Wilms' tumor through the effect of decreased IGF2R dosage on IGF-II inactivation, we examined IGF2R imprinting in Wilms' tumors. Two transcribed CA repeat polymorphisms were used to distinguish the two alleles in the RT-PCR product. We observed that in 7/16 of Wilms' tumor patients, the paternal IGF2R was markedly but not completely repressed in both tumor and normal kidney. In one additional case, IGF2R was likewise imprinted in the tumor but not in the normal kidney. A similar imprinting was observed in fetal tissues and placenta prior to 20 weeks fetal age but not in term placenta or postnatal blood cells, indicating abnormal persistence of a fetal pattern in the kidneys of Wilms' patients. Genetic analysis showed association of the imprinting with a cis-acting locus. The high frequency of aberrant persistence of IGF2R imprinting in the kidneys of Wilms' tumor patients, which may be an embryonic feature, suggests that it is a predisposing factor in tumorigenesis. This is in accordance with evidence that IGF2R is a tumour suppressor in other types of malignancies.

Alleles↗
PubMed1997

Molecular genetic pathways to Wilms tumor.

Wilms tumor remains a fascinating model for understanding how genes important in normal human embryogenesis can also contribute, in their mutant form, to cancer development in childhood. The cloning of the first Wilms tumor gene, WT1, in 1989, laid the framework for a model but also emphasized the underlying genetic complexity of this embryonal kidney cancer. Despite longstanding evidence for additional Wilms tumor gene loci, by 1997 very few of these have been cloned and none has yet been proven to be involved in Wilms tumorigenesis in man. However, the potential biological properties of these candidate genes suggest that disregulation of fetal mitogens, such as insulin-like growth factor 2, may be pivotal. Nephrogenesis is clearly sufficiently flexible to absorb many genetic errors. At present, it is unclear how often a simple two mutation model may account for Wilms tumor. Understanding how the various Wilms tumor genes interrelate, if indeed they do, awaits their identification. Piecing together these pathways may eventually lead to logical targets for therapeutic interventions.

Animals↗
PubMed1983

[Simultaneous bilateral Wilms' tumors].

Wilms' tumors occur in 4-14% bilaterally, partially simultaneously, partially metachronously. Therefore in every case with Wilms' tumor the contralateral kidney has to be examined thoroughly too. 5 children of a series of 52 patients with Wilms' tumor showed simultaneous bilateral occurrence (9,6%). The radiological procedures and their importance in diagnosis of bilateral Wilms' tumor are discussed. The prognosis of simultaneous bilateral Wilms' tumors is much better than that of metachronous ones and almost the same as that of unilateral ones.

Child, Preschool↗
PubMed1987

The in vitro growth, heterotransplantation, and immunohistochemical characterization of the blastemal component of Wilms' tumor.

Wilms' tumor has been proposed to originate from a developmental abnormality of the metanephric blastema. This undifferentiated component of Wilms' tumors has previously eluded efforts for in vitro growth. Blastema from a "classical" Wilms' tumor was transplanted into nude mice and passaged through 12 generations of heterotransplantation. Tumors from heterotransplants were grown for 12 serial passages in a serum-free growth medium supplemented with hormones and conditioned media from human kidney proximal tubule cells. The blastema initially grew on a collagen-fetal calf serum matrix as multicellular spheroids, and the cells proliferating from the rim of the spheroids had a flattened shape. Pulse-labeling with bromodeoxyuridine (BrdU) identified the proliferating cell population as blastemal in origin. Except for a loss of extracellular matrix, ultrastructural studies demonstrated morphologic similarities in the cultured cells, compared with the primary tumor and heterotransplants. Lectin histochemical stains for the peanut lectin (PNA) and immunohistochemical stains for cytokeratin (CYTO), vimentin (VIM), and epithelial membrane antigen (EMA) were performed on the original tumor, successive heterotransplants, and cells grown in vitro. The PNA stained the surface of the blastemal cells after sialidase digestion in the original tumor, heterotransplants, and cultured cells. The blastema of the original tumors was negative for CYTO and EMA but reactive for vimentin. This lack of differentiation was maintained in heterotransplants through 12 passages. However, blastemal cells demonstrated coexpression of CYTO and VIM intermediate filaments when grown in a serum-free medium on a matrix material. These studies demonstrate that the blastemal component of Wilms' tumor can be successfully grown in culture, passaged in nude mouse heterotransplants, and shown to undergo early stages of blastemal differentiation in vitro by growth in serum-free medium. This in vitro system provides a model for testing the factors that influence the growth and differentiation of the blastemal component of Wilms' tumors.

Animals↗
PubMed1994

A developmental context for multiple genetic alterations in Wilms' tumor.

Wilms' tumor has served as an example of Knudson's two-hit hypothesis of recessive tumor genes, but the genetics has proven to be surprisingly complex. WT1, a tumor suppressor gene on 11p13, is mutated in only a small fraction of Wilms' tumors, and a second chromosomal region, 11p15, harbors a second Wilms' tumor gene also involved in other cancers. In addition, loss of genomic imprinting, or parental origin-specific gene expression of at least two genes, appears to be an early step in Wilms' tumorigenesis and common cancers. Finally, genes on other chromosomes also play a role. I propose a model of Wilms' tumorigenesis in which multiple genetic alterations act within a specific developmental context, accounting for the epidemiological and pathological heterogeneity of Wilms' tumor, as well as the tissue specificity of the tumor types arising from alterations in these genes.

Beckwith-Wiedemann Syndrome↗
PubMed1993

Epidemiology of Wilms tumor.

Wilms tumor affects approximately one child per 10,000 worldwide before the age of 15 years. Incidence rates appear to be slightly elevated for U.S. and African Blacks in comparison to Whites, but are only half as great among Asians. Several case-control studies have suggested that paternal occupational or maternal hormonal exposures during pregnancy may increase the risk of Wilms tumor, but small numbers of subjects and inconsistencies in the patterns of exposures do not permit firm conclusions to be drawn. It is unlikely that such environmental exposures play a major role in the etiology of Wilms tumor. The median age-at-onset of Wilms tumor is 38 months in the U.S. National Wilms Tumor Study series, with cases in girls occurring on average 6 months later than in boys. Patients with bilateral tumors, aniridia, cryptorchism/hypospadias, Beck-with-Wiedemann syndrome, or intralobar nephrogenic rests tend to be diagnosed much younger than average (median 17-27 months). Those with familial disease or multicentric tumors have intermediate age-at-onset distributions, while those with perilobar nephrogenic rests are diagnosed at older ages. The epidemiologic features suggest that somatic mosaicism, rather than a germline mutation, may be responsible for some of the bilateral and multicentric cases.

Africa↗
PubMed1989

Spontaneous rupture of adult Wilms' tumor.

Wilms' tumor is uncommon in adults, and spontaneous retroperitoneal hemorrhage as the presenting sign of renal malignancy also is rare. A 22-year-old man with spontaneous rupture of a Wilms' tumor, who died within 5 months, is described. The factors that probably contributed to his rapid demise included preoperative tumor spilling, unfavorable histology, and the omission of radiotherapy owing to poor patient compliance. Although Wilms' tumor is known to have a poorer prognosis in adults than in children, the reason for this is not known. Due to the limited number of cases reported, the optimal treatment for adult Wilms' tumor remains to be defined. However, the recent literature indicates that radical surgery combined with maximal radiotherapy dosage and modification of the chemotherapy schedules used in children may be required to improve the prognosis for adults with Wilms' tumor.

Adult↗
PubMed1984

Management of unresectable Wilms tumor.

Wilms tumor has become a model of highly curable cancer. With the addition and refinement of chemotherapy protocols, survival rates have risen from 20 per cent three decades ago to 80 per cent or greater in the past decade. Surgery remains an integral part of the management of Wilms tumor both as a diagnostic tool and for removal of tumor bulk. Few primary tumors cannot be surgically removed during the initial phase of therapy. However, bilateral Wilms tumor and large unilateral tumors may occasionally be initially unresectable without prohibitive loss of renal or nonrenal organ function. The National Wilms Tumor Study group has no protocol for management of these tumors but does offer guidelines unsupported by large numbers of patients. Two cases are presented in which the tumors were initially deemed surgically unresectable. Initial management with chemotherapy (both cases) and radiotherapy (1 case) followed by surgical removal of tumor left both patients free of gross tumor, with relapse-free survivals of two and one-half years and six years thus far. Based on our experience and that found in the literature, we believe that preoperative chemotherapy is indicated for children suspected of having Wilms tumors judged surgically unresectable.

Child↗
PubMed1997

Wilms' tumor.

Wilms' tumor is the most common renal malignancy of childhood. Survival rates for favorable histology of Wilms' tumor currently approach 90%. Recent advances in the molecular genetics of Wilms' tumor have led to insights into the genetic factors involved in the development of Wilms' tumor, normal renal development, and the mechanisms of tumorigenesis.

Combined Modality Therapy↗