Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 Feb;60(2):136-139.
doi: 10.1016/j.ejmg.2016.12.001. Epub 2016 Dec 10.

Calcifying nested stromal-epithelial tumor (CNSET) of the liver in Beckwith-Wiedemann syndrome

Nasim Khoshnam  1 , Haynes Robinson  2 , Michael R Clay  1 , Lauren R Schaffer  1 , Scott E Gillespie  1 , Bahig M Shehata  3
Affiliations
Review

Calcifying nested stromal-epithelial tumor (CNSET) of the liver in Beckwith-Wiedemann syndrome

Nasim Khoshnam et al. Eur J Med Genet. 2017 Feb.

Abstract

Calcifying nested stromal-epithelial tumor (CNSET) is a rare neoplasm. In the 31 reported cases, CNSET is predominantly found in young girls and women. Beckwith-Wiedemann syndrome (BWS) (OMIM #130650) is an overgrowth syndrome with an increased risk to develop cancer. Associations have been seen between BWS and embryonal tumors, especially Wilms tumor, hepatoblastoma, and adrenocortical carcinoma. Here we report on a female patient with BWS who presented with CNSET. Two other cases with the same association have been reported, with our case representing the third such reported in the literature. Although we recognize a potential reporting bias we speculate that CNSET may represent an unrecognized addition to the spectrum of BWS tumorigenesis.

Keywords: Abnormal methylation at 11p15.5; Beckwith-Wiedemann syndrome (BWS); Calcifying nested stromal-epithelial tumor (CNSET); Uniparental disomy.

PubMed Disclaimer

LinkOut - more resources