期刊
PEDIATRIC BLOOD & CANCER
卷 64, 期 8, 页码 -出版社
WILEY
DOI: 10.1002/pbc.26432
关键词
11p overgrowth; adrenal cytomegaly; adrenal mass; Beckwith-Wiedemann syndrome; cancer predisposition; isolated hemihypertrophy; tumor screening
资金
- Alex's Lemonade Stand Foundation
- National Institutes of Health [K07 CA166177, K08 CA193915, T32HD043021-11A1]
- St. Baldrick's Foundation
Beckwith-Wiedemann syndrome (BWS) is a genetic overgrowth and cancer predisposition syndrome, associated with both benign and malignant adrenal findings. Literature review and an institutional case series elucidate the wide spectrum of adrenal findings in BWS patients. The altered expression of the 11p15 region is likely related to adrenal gland hyperplasia and growth dysregulation. Given the absence of guidelines for managing adrenal findings in BWS, we propose a systematic approach to adrenal findings in BWS patients, to allow for maximum detection of potentially malignant pathology without posing additional risk to patients.
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