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Evidence for Genetic Heterogeneity in Familial Wilms' Tumor¹

Departments of Experimental Pediatrics/Genetics [V. H., L. C. S., J. M. M.] and Epidemiology [C. I. A.], University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030; Department of Pediatric, Temple University School of Medicine, St. Christopher's Hospital for Children, Philadelphia, Pennsylvania 19134 [E. C. D.]; Department of Pathology and Tumor Cell Biology, St. Jude Children's Research Hospital, Memphis, Tennessee 38101 [R. F.]; Department of Pediatrics, University of Texas Health Science Center, San Antonio, Texas 78284 [C. F. G.]; Children's Hospital Medical Center of Akron, Akron, Ohio 44308-1062 [C. E. K.]; and Dana Farber Cancer Institute and Harvard School of Public Health, Boston, Massachusetts 02115 [F. P. L.]

Wilms' tumor (WT), a childhood kidney cancer, occurs both sporadically and, less frequently, in a familial context. Genetic linkage studies of several large WT families have excluded the one cloned WT gene, WT1, as the locus responsible for familial predisposition. These data demonstrate the existence of a familial predisposition gene distinct from WT1 and, more broadly, imply that the genetic etiology of WT is heterogenous. However, it has been unknown whether the predisposition observed in large WT families is also heterogenous or perhaps is due to mutations at a single locus. Recently, examination of a large French-Canadian WT family has demonstrated genetic linkage to 17q12–q21. We report here the results from a genetic linkage study of six WT pedigrees. Analyses of genotype data from eight loci within the 17q12–q21 region in these families resulted in cumulative lod scores of < -4.0 through the region, thereby excluding linkage. The ability to rule out the 17q region as the site of a predisposition gene in several of these pedigrees individually demonstrates the existence of more than one gene that predisposes to WT in large pedigrees and again emphasizes that the etiology of WT is genetically heterogenous.

¹ Supported by Grants CA34936 and CA60114.

² To whom requests for reprints should be addressed, at Department of Experimental Pediatrics/Genetics, Box 88, University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030. Phone: (713) 745-0678; Fax: (713) 792-4373; E-mail: vhuff@odin.mdacc.tmc.edu.

Received 2/27/97. Accepted 4/ 2/97.

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