Association of Wilms's Tumor with Aniridia, Hemihypertrophy and Other Congenital Malformations

Rw, Miller; Jf, Fraumeni; Manning,

doi:10.1056/nejm196404302701802

Cited by 560 publications

(173 citation statements)

References 19 publications

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“…20 Perhaps this indicates physicians are more aware of this association, and, therefore, report it more often.…”

Section: Discussionmentioning

confidence: 99%

Congenital anomalies in children with Wilms' Tumor. A new survey

Pendergrass

1976

Cancer

151

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From 68 hospitals i n the National Wilms' T u m o r Study, records of 547 patients showed six patients with aniridia, 16 with hemihypertrophy, and 24 with genitourinary abnormalities. Multiple cases of Wilms' tumor occurred in three families. The results confirm high frequencies of aniridia and genitourinary anomalies i n patients with Wilms' tumor, and show that concurrence with hemihypertrophy may be more often recognized or recorded now than it was ten years ago. T h e results demonstrate the desirability of developing checklists for other childhood neoplasms some of which have their own constellation of anomalies. This study was made possible by the cooperation of the investigators participating in the National Wilms' Tumoi Study. The author wishes to thank the National Wilms' Tumor Study Committee for the opportunity to review these data and to acknowledge the coopcration and assistance of the NWTS Data Center. Grateful appreciation is given for review of the manuwipt by Dr. Robert W. Miller and for the help of Mrs. Me1 Harren and Mrs. Jackie Pendergrass in abstracting and organizing the data.Received for publication January 22, 1975. MATERIALS AND METHODST h e NWTS was designed to refine the therapy of W T by using protocol treatment schedules in a national cooperative study.4 One of the secondary objectives of the study was to evaluate the epidemiology of W T . A checklist of those anomalies known to occur with W T was included on the physician's registration form for each patient. A second form was given to each family. This family questionnaire requested details of the health of the parents and siblings and a listing of any family member with cancer, tumors, kidney ailments, allergies, growths, and birth defects.T h e physician's registration forms were reviewed for each patient and the presence of any anomaly was abstracted. These data were correlated with those from the family questionnaires. Twenty patients who had incomplete checklists and no family questionnaires available and 28 cases whose diagnoses were changed from W T were excluded from the study. RES u LTS

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“…20 Perhaps this indicates physicians are more aware of this association, and, therefore, report it more often.…”

Section: Discussionmentioning

confidence: 99%

Congenital anomalies in children with Wilms' Tumor. A new survey

Pendergrass

1976

Cancer

151

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“…A small fraction of cases is associated with congenital aniridia (Miller et al, 1964), an association that was attributed to heterozygous deletion of both loci (Knudson & Strong, 1972). Such deletions were subsequently found and localised to chromosomal band 13q14 (Francke et al, 1979).…”

Section: Wilms' Tumour and Rhabdomyosarcomamentioning

confidence: 99%

The ninth Gordon Hamilton-Fairley memorial lecture: Hereditary cancers: clues to mechanisms of carcinogenesis

1989

Br J Cancer

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The study of hereditary cancer in humans, notably retinoblastoma, has identified a category of cancer genes that is different from that of the oncogenes. Whereas the latter group of genes exerts its effect through expression, the former does so as a result of failure of normal expression. Primary oncogene abnormality seems to play a crucial initiating role in certain neoplasms, particularly leukaemias, lymphomas and some sarcomas. In contrast, anti-oncogenes (tumour suppressor genes) appear to be important in the initiation of several solid tumours of children, as well as some common carcinomas of adults. Both classes are apparently involved in tumour progression and metastasis. Virtually every kind of cancer can occur in hereditary form, so the role of anti-oncogenes in the origin of human cancers may be considerable. The prototypic anti-oncogene has been that for retinoblastoma. For this tumour the recessive mechanism has been demonstrated by molecular means, and the gene has been cloned. The possibility has been suggested that gene (or gene product) replacement therapy could be accomplished.

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“…Wilms tumor-aniridia-genital anomalies-retardation syndrome (WAGR) called also 11p13 deletion Syndrome was first described by Miller et al [1]. It is a rare autosomal dominant disease, characterized by a de novo deletion within the distal band of chromosome 11, sub band 11p13 [2].…”

Section: Introductionmentioning

confidence: 99%