Simultaneous Presentation of Wilms Tumor and Immature Ovarian Teratoma in Beckwith-Wiedemann Syndrome. Issue 1 (January 2018)

• Record Type: Journal Article
• Authors: White, Jason C.; Liu, Jinglan; Nahar, Akash
• Is Part Of: Journal of pediatric hematology/oncology. Volume 40:Issue 1(2018)
• Publication Date: 2018-01
• Subjects: Wilms tumor -- immature ovarian teratoma -- Beckwith-Wiedemann syndrome -- H19 -- Igf-2; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; 618.9215
• DOI: 10.1097/MPH.0000000000000905
• Languages: English
• ISSNs: 1077-4114

Abstract

Abstract : The Beckwith-Wiedemann syndrome is a cancer predisposition syndrome characterized by a predilection to embryonal tumor growth, especially Wilms tumor, adrenocortical carcinomas, and hepatoblastomas. Genetic analysis of patients has revealed a link to the imprinted domain of the 11p15.5 chromosome and methylation status of the H19 locus and Igf-2 . These genes have also been studied in other cancers, including ovarian teratomas. Our case is a patient with a simultaneous presentation of a Wilms tumor and immature ovarian teratoma and subsequently diagnosed with Beckwith-Wiedemann syndrome, which has not been previously described.