Work

Management of adrenal masses in patients with Beckwith–Wiedemann syndrome

Year: 2017

Type: review

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.

Source: Pediatric Blood & Cancer

DOI: 10.1002/pbc.26432

Authors: Suzanne P. MacFarland, Sogol Mostoufi‐Moab, Kristin Zelley, Peter A. Mattei, Lisa J. States, Tricia R. Bhatti, Kelly A. Duffy, Garrett M. Brodeur, Jennifer M. Kalish

Institutions: Children's Hospital of Philadelphia, University of Pennsylvania

Language: English

Topic: Genetic Syndromes and Imprinting

Subfield: Genetics

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: green

Funders: Alex's Lemonade Stand Foundation for Childhood Cancer, St. Baldrick's Foundation, National Institutes of Health, National Cancer Institute, Eunice Kennedy Shriver National Institute of Child Health and Human Development

Awards: The Mechanism of Tumor Formation in Beckwith-Wiedemann Syndrome, Bone Deficits and Excess Adiposity after Pediatric Bone Marrow Transplantation, CHOP Institutional Training in Pediatric Research

OpenAlex ID: W2567964787

FWCI: 0.457

Cites: 54

Cited by: 33

Related to: 10