Occurrence of nephroblastomatosis with dup(18)(q11.2-q23) implicates trisomy 18 tumor screening protocol in select patients with 18q duplication

Am J Med Genet A. 2014 Apr;164A(4):1079-82. doi: 10.1002/ajmg.a.36432. Epub 2014 Mar 4.

Abstract

Duplications of the long arm of chromosome 18 have been previously reported in patients with phenotypic findings similar to full trisomy 18. Trisomy 18 increases the risk for Wilms tumor and it is currently recommended that these patients undergo abdominal ultrasonography screening every 6 months. We report on nephroblastomatosis in a 27-month-old male with a 55 Mb duplication of chromosome 18q11.2-q23 (chr18:22693370-77982126, hg 19) and propose that the trisomy 18 tumor screening protocol could also benefit patients with large 18q duplications.

Keywords: 18q duplication; Wilms tumor; nephroblastomatosis; partial trisomy 18; trisomy 18.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Chromosomes, Human, Pair 18* / genetics
  • Fetal Macrosomia / genetics*
  • Gene Duplication*
  • Humans
  • Male
  • Trisomy / genetics*
  • Trisomy 18 Syndrome
  • Wilms Tumor / genetics*

Supplementary concepts

  • Chromosome 18, trisomy 18q
  • Nephroblastomatosis, fetal ascites, macrosomia and Wilms tumor