Association of Alveolar Rhabdomyosarcoma with the Beckwith-Wiedemann Syndrome

  title={Association of Alveolar Rhabdomyosarcoma with the Beckwith-Wiedemann Syndrome},
  author={Adam C. Smith and Jeremy Andrew Squire and Paul Scott Thorner and Maria Zielenska and Cheryl T. Shuman and Ronald M Grant and David Chitayat and Joy L. Nishikawa and Rosanna Weksberg},
  journal={Pediatric and Developmental Pathology},
Rhabdomyosarcoma (RMS) is a soft tissue tumor of childhood frequently diagnosed between the first and fifth year of life. Children with the Beckwith-Wiedemann syndrome (BWS), a congenital overgrowth syndrome characterized by exomphalos, macroglossia, and macrosomia, have an increased risk of developing childhood tumors including Wilms tumor, hepatoblastoma, neuroblastoma, and RMS. Although an association between RMS and the BWS is well accepted, only four cases have been reported to date, and… 

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The present work was aimed at reviewing the cytogenetic alterations observed in the different histological subtypes of RMS, focusing not only on the studies performed with conventional cytogenetics, but also on new approaches used in the study of neoplasms, such as FISH, CGH, SKY and M-FISH.

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    Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
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  • 1995
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