| Introduction to the genetics of primary renal tumors in children. | |
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MedLine Citation:
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PMID: 8383279 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Wilms tumor can be explained only partially by the "two hit" model that was originally developed for retinoblastoma. Heterogeneity of two kinds operates. The first is that four other primary tumors are regularly observed in children, and the second is that Wilms tumor itself appears to represent more than one genetic entity. All five of these primary renal tumors arise from primary or secondary mesenchyme, renal blastema, or renal epithelium. Mesoblastic nephroma, and possibly clear cell sarcoma, may have some genetic affinity with Wilms tumor, but rhabdoid tumor of the kidney and renal carcinoma do not. At least three different genes seem to be important in the origin of Wilms tumor. One, WT1, whose mutations may be associated with aniridia, may follow the "two hit" model in that there are cases in which both copies of the gene are defective or lost, as expected for a tumor suppressor gene. A second gene, which is associated with Beckwith-Wiedemann Syndrome (BWS) and which has not been cloned, appears to be imprinted in females, and may have an oncogene function. It is evidently activated by gain of a paternal allele or by loss of the inactive, but possibly trans-sensing, maternal allele. Activation of the insulin-like growth factor II gene may be a final common pathway for mutation in both WT1 and BWS. A third gene is unlinked to either of the other two, but its location and function are unknown. It shares with WT1 specificity for Wilms tumor, which is not true of the BWS gene. |
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Authors:
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A G Knudson |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Review |
Journal Detail:
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Title: Medical and pediatric oncology Volume: 21 ISSN: 0098-1532 ISO Abbreviation: Med. Pediatr. Oncol. Publication Date: 1993 |
Date Detail:
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Created Date: 1993-04-05 Completed Date: 1993-04-05 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 7506654 Medline TA: Med Pediatr Oncol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 193-8 Citation Subset: IM |
Affiliation:
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Institute for Cancer Research, Fox Chase Cancer Center, Philadelphia, PA 19111. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Child Genes, Wilms Tumor Humans Kidney Neoplasms / genetics*, pathology Syndrome Wilms Tumor / genetics*, pathology |
| Grant Support | |
ID/Acronym/Agency:
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CA-06927/CA/NCI NIH HHS; R01 CA 43211/CA/NCI NIH HHS |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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