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.

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

MacFarland SP,Mostoufi-Moab S,Zelley K,Mattei PA,States LJ,Bhatti TR,Duffy KA,Brodeur GM,Kalish JM

doi

10.1002/pbc.26432

subject

Has Abstract

pub_date

2017-08-01 00:00:00

issue

8

eissn

1545-5009

issn

1545-5017

journal_volume

64

pub_type

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