Calcifying nested stromal-epithelial tumor (CNSET) of the liver: a newly recognized entity to be considered in the radiologist's differential diagnosis.

Calcifying nested stromal-epithelial tumor (CNSET), an extremely rare tumor found in the liver, was first described in 2001 by Ishak et al. The characteristic imaging features include large size, well-circumscribed, enhancing mass with calcification. To our knowledge, since 2001, there have been 29 reported. Typically arising from the right hepatic lobe, it is primarily found in children and shows clear predilection for females. Emphasizing imaging, we report a 14-year-old female with Beckwith-Wiedemann syndrome who presented with CNSET.