Article Text

Download PDFPDF
Costello syndrome: two cases with embryonal rhabdomyosarcoma.
  1. B Kerr,
  2. O B Eden,
  3. R Dandamudi,
  4. N Shannon,
  5. O Quarrell,
  6. A Emmerson,
  7. E Ladusans,
  8. M Gerrard,
  9. D Donnai
  1. North West Regional Genetic Service, St Mary's Hospital, Manchester, UK.


    Costello syndrome is a well delineated mental retardation syndrome of unknown aetiology in which the occurrence of benign tumours, especially papillomata, is recognised. We report two children in whom the diagnosis of Costello syndrome was made in the first months of life, who both developed a retroperitoneal embryonal rhabdomyosarcoma. Although not previously reported, the occurrence of this relatively uncommon childhood tumour in two girls with Costello syndrome suggests that an increased risk of malignancy may be part of this condition. The genetic basis of this susceptibility requires further clarification.

    Statistics from

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    Linked Articles