Hepatic Adenoma Arising in a Patient With Alagille Syndrome: A Case Report.

Pediatr Dev Pathol

5 Department of Pediatrics, University of Washington, Seattle, Washington.

Published: December 2018

Alagille syndrome is associated with decreased bile ducts, cardiac abnormalities, vertebral body fusion defects, and a typical facies. While regenerative nodules and hepatocellular carcinoma have been described in these patients, hepatic adenoma has not. Herein, we present a patient with Alagille syndrome caused by a mutation in NOTCH2 with a hepatic adenoma. The clinical, imaging, and histologic features are discussed.

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http://dx.doi.org/10.1177/1093526618762550DOI Listing

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