Bilateral nephroblastoma associated with a 3;17 translocation

Soulie, Joëlle; Rousseau-Merck, Marie-Françoise; Mouly, Hélène; Nézelof, C

doi:10.1159/000132104

Cited by 5 publications

(1 citation statement)

References 7 publications

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“…A compilation of the cytogenetic rearrangements published over 10 years for 107 sporadic W T (Slater and d e Kraker, 1982;Ferrell et al, 1983;Kaneko et al, 1983;Kondo et al, 1984;Douglass et al, 1985;McGavran et al, 1985;Slater et al, 1985;Soulie et al, 1985;Douglass et al, 1986; Slater, 1986;Tsai et al, 1987;Wolman et a]., 1987;Solis et al, 1988;McDowel et al, 1989;Vekemans et al, 1989;Wang-Wuu et al, 1990) showed that rearrangements of chromosomes 1, 11, 12, and 16 appeared to occur at similar frequencies, with the most common being dup( 12) and del( 16q) ( Table 1, Fig. 1).…”

Section: Sporadic Wtmentioning

confidence: 99%

Characterization of regions of chromosomes 12 and 16 involved in nephroblastoma tumorigenesis

Austruy

Candon

Henry

et al. 1995

Genes Chromosomes & Cancer

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There are at least three loci involved in Wilms' tumor (WT) tumorigenesis: WT1 in 11p13, WT2 in 11p15.5, and WT3, as yet unmapped. A compilation of cytogenetic data published for 107 WT revealed that deletion of chromosome 16 and duplication of chromosome 12 occur as frequently as the well-documented 11p deletions. Allelic imbalance for chromosomes 16 and 12 was investigated in a series of 28 WT. By use of a large panel of restriction fragment length polymorphisms and (CA)n probes, we demonstrated loss of heterozygosity (LOH) for 16q in seven (25%) of the tumors. The whole length of 16q was involved in six of the tumors. Moreover, consistent with a previous report of 16q13 LOH in a sporadic WT and a constitutional breakpoint with a Beckwith-Wiedemann patient, we map a region of particular interest to between D16S308 and D16S320. The assumption that 16q LOH may be an early event was based on: 1) the detection of 16q LOH in one case of nephroblastomatosis; 2) the presence of a complete (clonal) 16q LOH in a tumor with partial (mosaic) 11p LOH; and 3) 16q LOH as the sole abnormality in one WT. By quantification of chromosome 12 allelic imbalance, we detected duplication in 18% of the total series and in 25% of the sporadic unilateral cases. The common region extended from the centromere to D12S7 in 12q21.1-q23. We also suggest that the various pathogenetically important loci are not equally involved in the different forms of WT and that their sequential involvement may differ.

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Section: Sporadic Wtmentioning

confidence: 99%

Characterization of regions of chromosomes 12 and 16 involved in nephroblastoma tumorigenesis

Austruy

Candon

Henry

et al. 1995

Genes Chromosomes & Cancer

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Progression of Embryonal and Mixed Neoplasms

Kaiser¹

1989

Local Invasion and Spread of Cancer

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Wilms' tumor ? treatment and prognosis of 373 children

et al. 1992

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Between June 1980 and June 1988, 373 patients with Wilms' tumors were diagnosed and treated (34.5%, stage I; 34.5%, stage II; 14.8%, stage III; 10.7%, stage IV; and 5.4%, stage V). In all, 11% of the patients were diagnosed as a result of routine checkup examinations. The tumor volume was < 400 ml in 47.3% of cases and > 4 0 0 m l in 52.7%. Overall, 78.7°7o of the patients had a standard-type Wilms' tumor, 7% had a low-grade malignancy lesion and 14.3% had a high-grade-malignancy tumor. Aside from radical tumor nephrectomy, treatment encompassed chemotherapy in all cases and radiotherapy in half of the patients according to the study protocol. In all, 32% of the children received preoperative treatment and 68% underwent primary surgery. Overall, 81.8070 (305/373) of the patients were disease-free according to the probability of disease-free survival after 6 years as calculated using the Kaplan-Meier method, with 90O7o of the protocol group (196/218) being free of disease. After primary surgery and subsequent chemotherapy, 97.3% of the children with standard-type Wilms' tumors of stage I were cured and 94°7o of those with stage II tumors survived disease-free, as did 88% of those in stage III, 46°7o of those in stage IV, and 75O7o of those in stage V (Kaplan-Meier method). Radiotherapy was performed in 113 of the 218 protocol patients. Children demonstrating a clear-cell histology subtype did not show the poor prognosis attributed to this histology in the literature. Of the children with low-grade variants, 19 of 24 are free of disease. About 70% of all children with Wilms' tumor in the Federal Republic of Germany were entered in this study (47 of probably 68 new cases recorded annually nationwide x 8 years). Of the 47 patients evaluated per year, 38 survived disease-free, whereas 9 per year either suffered from a disease or perished. The overall results are very satisfying. Additional treatment modifications can be achieved only through further studies. Not only for this reason is it recommended that Wilms' tumors be treated at large institutions.* To whom correspondence should be addressed Wilms' tumor is the most common solid childhood tumor; 6.5% of all malignancies occurring in children are nephroblastomas. About 70 new cases are diagnosed every year in the Federal Republic of Germany.The therapy of Wilms' tumors depends on various factors such as the clinical stage and histologic subtype of the tumor and the age of the patient. New information for optimization of the therapy can only be gathered by means of a nationwide study. We report on the clinical data and treatment results of 373 children. The aim of this study was to elucidate the relationship between the prognosis and the clinical stage and histologic subtype of the tumor as well as the age of the patients. In addition, the importance of pre-and/or postoperative radiotherapy was studied. Furthermore, the dose and duration of treatment with chemotherapeutic agents was evaluated. Patients and methods SurgeryChildren aged <2 years received primary surge...

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Bilateral nephroblastoma associated with a 3;17 translocation

Cited by 5 publications

References 7 publications

Characterization of regions of chromosomes 12 and 16 involved in nephroblastoma tumorigenesis

Characterization of regions of chromosomes 12 and 16 involved in nephroblastoma tumorigenesis

Progression of Embryonal and Mixed Neoplasms

Wilms' tumor ? treatment and prognosis of 373 children

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