Beckwith-Wiedemann Syndrome (BWS)
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BWS arises from epigenetic changes or genetic mutations in genes that regulate growth, particularly on chromosome 11p15. This region contains genes such as IGF2 and H19, which play a crucial role in cell proliferation.
ACC occurs in association with BWS but is significantly rarer than other tumors. Children with BWS have an increased risk of developing tumors, including ACC, particularly in early childhood. Therefore, close monitoring is recommended during the first 8 years of life.