Wilms tumor in monozygous twins: Clinical, pathological, cytogenetic and molecular case report

Daniela Perotti, Giovanna De Vecchi, Elena Lualdi, M. Adele Testi, Gabriella Sozzi, Paola Collini, Filippo Spreafico, Monica Terenziani, Franca Fossati-Bellani, Paolo Radice

Research output: Contribution to journalArticle

Abstract

The concomitant occurrence of Wilms tumor (WT) was observed in two monozygotic twin sisters without evidence of congenital malformations. Twin 1 was diagnosed with a stage I WT at 11 months of age, whereas twin 2 developed a bilateral (stage V) WT at 13 months of age. In both cases pathologic examination showed a nonanaplastic stromal type WT, with marked rhabdomyomatous elements. Cytogenetic analyses performed on blood samples and on tumor specimens revealed no karyotypic abnormality. No alteration of the WT1 and POU6F2 genes was identified in constitutional and tumor DNA of both sisters, and no anomaly in WT1 expression was evidenced in the normal kidney of one of them. However, loss of heterozygosity on chromosome 11p, involving the alleles of maternal origin, was detected both in the single tumor of twin 1 and in the two distinct tumors of twin 2, thus suggesting a common etiology of the diseases. To the authors' knowledge, this is the first report describing at both the clinical and genetic level a couple of monozygotic twins concordant for WT development.

Original languageEnglish
Pages (from-to)521-525
Number of pages5
JournalJournal of Pediatric Hematology/Oncology
Volume27
Issue number10
DOIs
Publication statusPublished - Oct 2005

Fingerprint

Wilms Tumor
Cytogenetics
Monozygotic Twins
Siblings
Neoplasms
Loss of Heterozygosity
Cytogenetic Analysis
Chromosomes
Alleles
Mothers
Kidney
DNA
Genes

Keywords

  • Loss of heterozygosity
  • Monozygotic twins
  • POU6F2
  • Wilms tumor
  • WT1

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Hematology

Cite this

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title = "Wilms tumor in monozygous twins: Clinical, pathological, cytogenetic and molecular case report",
abstract = "The concomitant occurrence of Wilms tumor (WT) was observed in two monozygotic twin sisters without evidence of congenital malformations. Twin 1 was diagnosed with a stage I WT at 11 months of age, whereas twin 2 developed a bilateral (stage V) WT at 13 months of age. In both cases pathologic examination showed a nonanaplastic stromal type WT, with marked rhabdomyomatous elements. Cytogenetic analyses performed on blood samples and on tumor specimens revealed no karyotypic abnormality. No alteration of the WT1 and POU6F2 genes was identified in constitutional and tumor DNA of both sisters, and no anomaly in WT1 expression was evidenced in the normal kidney of one of them. However, loss of heterozygosity on chromosome 11p, involving the alleles of maternal origin, was detected both in the single tumor of twin 1 and in the two distinct tumors of twin 2, thus suggesting a common etiology of the diseases. To the authors' knowledge, this is the first report describing at both the clinical and genetic level a couple of monozygotic twins concordant for WT development.",
keywords = "Loss of heterozygosity, Monozygotic twins, POU6F2, Wilms tumor, WT1",
author = "Daniela Perotti and {De Vecchi}, Giovanna and Elena Lualdi and Testi, {M. Adele} and Gabriella Sozzi and Paola Collini and Filippo Spreafico and Monica Terenziani and Franca Fossati-Bellani and Paolo Radice",
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AU - Perotti, Daniela

AU - De Vecchi, Giovanna

AU - Lualdi, Elena

AU - Testi, M. Adele

AU - Sozzi, Gabriella

AU - Collini, Paola

AU - Spreafico, Filippo

AU - Terenziani, Monica

AU - Fossati-Bellani, Franca

AU - Radice, Paolo

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N2 - The concomitant occurrence of Wilms tumor (WT) was observed in two monozygotic twin sisters without evidence of congenital malformations. Twin 1 was diagnosed with a stage I WT at 11 months of age, whereas twin 2 developed a bilateral (stage V) WT at 13 months of age. In both cases pathologic examination showed a nonanaplastic stromal type WT, with marked rhabdomyomatous elements. Cytogenetic analyses performed on blood samples and on tumor specimens revealed no karyotypic abnormality. No alteration of the WT1 and POU6F2 genes was identified in constitutional and tumor DNA of both sisters, and no anomaly in WT1 expression was evidenced in the normal kidney of one of them. However, loss of heterozygosity on chromosome 11p, involving the alleles of maternal origin, was detected both in the single tumor of twin 1 and in the two distinct tumors of twin 2, thus suggesting a common etiology of the diseases. To the authors' knowledge, this is the first report describing at both the clinical and genetic level a couple of monozygotic twins concordant for WT development.

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