Management of adrenal masses in patients with Beckwith-Wiedemann syndrome.

TitleManagement of adrenal masses in patients with Beckwith-Wiedemann syndrome.
Publication TypeJournal Article
Year of Publication2017
AuthorsMacFarland SP, Mostoufi-Moab S, Zelley K, Mattei PA, States LJ, Bhatti TR, Duffy KA, Brodeur GM, Kalish JM
JournalPediatr Blood Cancer
Date Published2017 Jan 09
ISSN1545-5017
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.

DOI10.1002/pbc.26432
Alternate JournalPediatr Blood Cancer
PubMed ID28066990