Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0178874 (tumor progression)
 40,807 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Wilms tumor suppressor gene wt1 encodes a zinc finger DNA binding protein, WT1, that functions as a transcriptional repressor. The fetal mitogen insulin-like growth factor II (IGF-II) is overexpressed in Wilms tumors and may have autocrine effects in tumor progression. The major fetal IGF-II promoter was defined in transient transfection assays as a region spanning from nucleotides -295 to +135, relative to the transcription start site. WT1 bound to multiple sites in this region and functioned as a potent repressor of IGF-II transcription in vivo. Maximal repression was dependent on the presence of WT1 binding sites on each side of the transcriptional initiation site. These findings provide a molecular basis for overexpression of IGF-II in Wilms tumors and suggest that WT1 negatively regulates blastemal cell proliferation by limiting the production of a fetal growth factor in the developing vertebrate kidney.
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PMID:Repression of the insulin-like growth factor II gene by the Wilms tumor suppressor WT1. 132 41

Thin-layer chromatography was used to measure free polyamine level in blood plasma and peripheral blood cells in 38 pediatric nephroblastoma patients and 30 apparently healthy children. Thirty-six nephroblastoma patients were examined in the course of treatment of whom 25-during 1-36 months following basic treatment course as well. A significant increase in the initial free polyamine level in blood cells (92.4% of cases) and plasma (63%) of nephroblastoma patients was observed. A correlation was established between changes in free polyamine level, on the one hand, and response to treatment and subsequent course of disease, on the other. At 8-10 days following radical surgery, a decrease in polyamine level was observed in 58% of operated cases and an increase-in 41.8%. The latter proved prognostically unfavorable. Recurrence and metastases development was associated with a rise in polyamine concentration in plasma and, particularly, in blood cells which in some cases preceded the appearance of other signs of tumor progression. The polyamine test can be recommended for monitoring pediatric nephroblastoma patients.
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PMID:[The possibilities of using the free polyamines of the peripheral blood as biochemical tumor markers in nephroblastoma in children]. 133 69

One of the most exciting areas of molecular oncology is the convergence of two independent lines of evidence suggesting involvement of multiple tumor suppressor genes in a given type of cancer. First, epidemiology and somatic cell genetics indicate the presence of multiple tumor suppressor genes in each of several malignancies. Second, cancers often lose multiple chromosomal regions during tumor progression. We will use two tumors, colorectal cancer and Wilms tumor, to illustrate the questions that multiple tumor suppressor genes raise.
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PMID:Multiple tumor suppressor genes in multistep carcinogenesis. 133 99

The pathological and cytogenetic features of an extrarenal malignant rhabdoid tumor (MRT) arising from the paravertebral region in an infant were investigated. The patient died 4 months after diagnosis, due to aggressive tumor progression. The tumor was composed of medium-sized round cells with cytoplasm containing eosinophilic inclusions, which ultrastructurally were composed of densely packed whorled intermediate filaments. Flow-cytometric analysis of the tumor cells revealed a diploid pattern. Amplification of the N-myc oncogene was not identified. Immunohistologically, the inclusion bodies showed a positive reaction with antiserum against vimentin. The tumor cells were not reactive with antiserum against epithelial membrane antigen, anti-keratin (polyclonal) or cytokeratin (monoclonal, CK1), but did react with 5H10, an antiserum established from human sarcomatous Wilms' tumor. This case is discussed with reference to the literature on extrarenal MRT, placing stress on the histogenesis of this tumor.
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PMID:Cytogenetic characteristics of a malignant rhabdoid tumor arising from the paravertebral region. A case report. 150 7

Cell adhesion molecules (CAMs) of the immunoglobulin supergene family may play important roles in tumorigenesis and the development of metastatic disease. In a variety of human malignancies, tumor progression has been observed to be associated with changes in CAM expression. An early event in colorectal tumorigenesis appears to be the down regulation of a normally expressed CAM, DCC. Over-expression of a second CAM, carcinoembryonic antigen, is associated with colorectal tumors which have a high risk for metastasis development. Several tumors, including Wilms tumors and neuroblastoma, have been found to express a developmentally regulated form of NCAM which inhibits a variety of cell-cell interactions. Malignant cells not only show aberrations in the expression of their CAMS and thus their normal cell-cell interactions, but establish new adhesive interactions. The development of metastatic potential in cutaneous melanoma is associated with the de novo expression of two CAMs, one of which is ICAM-1, a molecule mediating adhesion between the tumor cells and leukocytes.
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PMID:Cell adhesion molecules of the immunoglobulin supergene family and their role in malignant transformation and progression to metastatic disease. 168 May 75

The curing chance of cancer disseminated to the lungs depends on the global curing chance of that specific tumor, the extent and distribution of its systemic spread and the availability of additional treatment modalities besides surgery. Of all tumors occurring in childhood and adolescence only osteosarcoma, Wilms tumor and Ewing's sarcoma preferentially disseminate to the lungs and such are the most promising candidates for successful treatment. In osteosarcoma with pulmonary dissemination surgical removal of the metastases is indispensable. In Wilms tumor chemoradiotherapy may replace or be used as an adjunct to surgery while in Ewing's sarcoma with primary pulmonary metastases chemoradiotherapy is the treatment of choice. Although metachronous lung metastases may still cured in osteosarcoma and Wilms tumor, they tend to be fatal however in Ewing's sarcoma. A small chance of success itself should not contraindicate metastasectomy but only the actual technically impossible intervention or the definite demonstration of tumor progression no longer controllable of different location. However, even palliative metastasectomy may be indicated in an individual patient.
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PMID:Surgical treatment of pulmonary metastases in childhood. 243 85

We have compared constitutional and tumor genotypes in nine cases of hereditary Wilms tumor (WT) and in three unrelated cases of familial adrenocortical carcinoma (ADCC). Since susceptibility to these tumors can be observed in malformation syndromes associated with a constitutional deletion of band 11p13 (WT) and with a constitutional duplication of band 11p15.5 (WT, ADCC), we investigated these two candidate regions by using 11p polymorphic markers. As expected, somatic chromosomal events, resulting in a loss of heterozygosity limited to region 11p15.5, were observed in the tumor of two familial cases of adrenocortical carcinoma. Surprisingly, however, analysis of the WT of two patients with a constitutional deletion of band 11p13, associated with aniridia, genitourinary abnormalities, and mental retardation (WAGR syndrome), revealed a loss of heterozygosity limited to region 11p15.5. These data therefore suggest that observation of a specific loss of heterozygosity may not necessarily point to the site of the initial germinal mutation. Together with previous similar observations of a loss of heterozygosity limited to 11p15.5 in breast cancer and in rhabdomyosarcoma, our data suggest that region 11p15.5 may carry a non-tissue-specific gene that could be involved in genetic predisposition, in tumor progression, or in both.
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PMID:Tumor-specific loss of 11p15.5 alleles in del11p13 Wilms tumor and in familial adrenocortical carcinoma. 256 68

Cellular oncogenes (c-oncs) have been highly conserved throughout evolution and subserve important roles in growth and development. Both in development and the neoplastic state, c-oncs appear to collaborate rather than function independently. Cellular oncogenes are activated in the neoplastic process by four (nonviral) mechanisms; (a) chromosomal translocations; (b) gene amplifications; (c) point mutations; and (d) DNA rearrangements. The timing of c-onc gene product expression may be as important in oncogenesis as the level of expression. At this writing, mutant oncogenes have not been shown to be inherited. Oncogene amplification, if important in oncogenesis, is more likely to be involved with tumor progression rather than initiation. Chromosomal/molecular aberrations tend to be characteristic for a given type of cancer. These genetic alterations are often situated near heritable fragile sites, tumor-suppressor gene loci and/or oncogene loci. Similar molecular mechanisms involving translocations and inversions may underly the common T and B cell neoplasms. The loss/inactivation of both normal alleles at a locus thought to encode for tumor-suppressing activities (antioncogenes) may represent an event common to many childhood and adult neoplasms. The consistency and cell specificity with which this has been identified is consistent with a role for such genes in cellular differentiation. At this writing, the paradigm for such a controlling locus is 13q14, the site of the retinoblastoma gene. Based on recent studies in familial and sporadic Wilms' tumor which suggest etiological heterogeneity, theoretical modifications of the carcinogenesis model which has been central to understanding retinoblastoma may soon be forthcoming to explain molecular mechanisms operative in other cancer. The role of genomic imprinting in carcinogenesis is only recently being explored. Further study of this process may prove to be a fruitful area of future research.
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PMID:Molecular mechanisms of oncogenesis. 265 71

The paper discusses the end results of complex treatment of 31 children comprising preoperative therapy, nephrectomy and adjuvant chemotherapy. Two-year survival was 64.5% and two-year recurrence-free survival--48.4%. Adjuvant chemotherapy (vincristine, dactinomycin and adriamycin) was given to 25 cases. Complete treatment consisting of 4 courses of adjuvant chemotherapy was carried out in 12 patients only, tumor progression and toxic hepatitis being the most frequent causes of adjuvant treatment suspension. Survival was shown to depend on such prognostic factors as the efficacy of preoperative chemotherapy, stage and morphological pattern of tumor rather than adjuvant chemotherapy duration. Therefore, a short course of adjuvant chemotherapy may be recommended for localized nephroblastoma in pediatric patients.
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PMID:[Adjuvant chemotherapy in the combined modality therapy of nephroblastoma in children]. 284 46

There were 122 deaths among 803 children registered, randomized, and followed in the second National Wilms' Tumor Study; 17 occurred in children apparently free of disease and were attributable to causes other than tumor progression. Seven deaths were attributed to infection during periods of drug-induced leukopenia; four were due to liver failure; and one each was attributable to radiation pneumonopathy, intestinal obstruction, renal failure, myocardial disease, and encephalopathy. The cause of one death was unexplained. Of particular concern were four (of 47) infants under one year of age with group I or II disease who had toxic deaths. Subsequent to these experiences the doses of all chemotherapeutic agents were reduced by 50% for infants under one year of age. No deaths from toxicity were observed thereafter in infants. An analysis of the therapeutic effect of this dose reduction showed three of 47 relapsed on full dose and five of 54 on half dose. The difference is not statistically significant. This report is a further demonstration of the potentially serious vulnerability of infants to standard doses of anticancer drugs even when they are calculated on a per kilogram basis.
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PMID:Toxic deaths in the Second National Wilms' Tumor Study. 608 9


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