2020
DOI: 10.1038/s41416-020-0911-x
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Cancer incidence and spectrum among children with genetically confirmed Beckwith-Wiedemann spectrum in Germany: a retrospective cohort study

Abstract: Background Beckwith-Wiedemann syndrome (BWS) is a cancer predisposition syndrome caused by defects on chromosome 11p15.5. The quantitative cancer risks in BWS patients depend on the underlying (epi)genotype but have not yet been assessed in a population-based manner. Methods We identified a group of 321 individuals with a molecularly confirmed diagnosis of BWS and analysed the cancer incidence up to age 15 years and cancer spectrum by matching thei… Show more

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Cited by 40 publications
(32 citation statements)
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“…They were found in Li-Fraumeni syndrome [ 87 , 93 , 94 ], multiple endocrine neoplasia type 1 [ 95 ], neurofibromatosis type 1 [ 96 ] and familial adenomatous polyposis [ 97 ]. In addition, paediatric ACCs were found in Beckwith–Wiedemann syndrome [ 98 , 99 ].…”
Section: Paediatric Adrenocortical Carcinomamentioning
confidence: 99%
“…They were found in Li-Fraumeni syndrome [ 87 , 93 , 94 ], multiple endocrine neoplasia type 1 [ 95 ], neurofibromatosis type 1 [ 96 ] and familial adenomatous polyposis [ 97 ]. In addition, paediatric ACCs were found in Beckwith–Wiedemann syndrome [ 98 , 99 ].…”
Section: Paediatric Adrenocortical Carcinomamentioning
confidence: 99%
“…In cases of overgrowth syndromes, organ growth may be overall symmetric, or localized to a specific area of the body. Many of these syndromes are associated with increased risk of cancer 10,11 . Prenatal diagnosis of visceromegaly involving not only the kidneys but also the pancreas provides valuable information that may drive targeted genetic analysis of overgrowth syndromes.…”
Section: Discussionmentioning
confidence: 99%
“…Papulino suggested there is an increased incidence of childhood tumor predisposition in BWS patients [9]. Cöktü [23] identified a group of 321 individuals with a molecularly confirmed diagnosis of BWS and analysed the cancer incidence. They confirmed an increased cancer risk in children with BWS.…”
Section: Discussionmentioning
confidence: 99%