title: Management of adrenal masses in patients with Beckwith-Wiedemann syndrome.
abstract: 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.
authors.affiliation: Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Suzanne P
authors.initials: SP
authors.lastname: MacFarland
authors.affiliation: Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.|Department of Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Sogol
authors.initials: S
authors.lastname: Mostoufi-Moab
authors.affiliation: Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Kristin
authors.initials: K
authors.lastname: Zelley
authors.affiliation: Department of Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.|Department of General, Thoracic, and Fetal Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Peter A
authors.initials: PA
authors.lastname: Mattei
authors.affiliation: Department of Radiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Lisa J
authors.initials: LJ
authors.lastname: States
authors.affiliation: Department of Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.|Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Tricia R
authors.initials: TR
authors.lastname: Bhatti
authors.affiliation: Division of Human Genetics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Kelly A
authors.initials: KA
authors.lastname: Duffy
authors.affiliation: Division of Oncology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.|Department of Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.
authors.forename: Garrett M
authors.initials: GM
authors.lastname: Brodeur
authors.affiliation: Department of Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.|Division of Human Genetics, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
authors.forename: Jennifer M
authors.initials: JM
authors.lastname: Kalish
mesh_terms: D000307:Adrenal Gland Diseases / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy*; D001506:Beckwith-Wiedemann Syndrome / Q000150:complications*; D006801:Humans
keywords: 11p overgrowth; Beckwith-Wiedemann syndrome; adrenal cytomegaly; adrenal mass; cancer predisposition; isolated hemihypertrophy; tumor screening