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

Authors :
Pacheco MC
Monroe EJ
Horslen SP
Source :
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society [Pediatr Dev Pathol] 2018 Nov-Dec; Vol. 21 (6), pp. 585-589. Date of Electronic Publication: 2018 Mar 08.
Publication Year :
2018

Abstract

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.

Details

Language :
English
ISSN :
1615-5742
Volume :
21
Issue :
6
Database :
MEDLINE
Journal :
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
Publication Type :
Academic Journal
Accession number :
29516774
Full Text :
https://doi.org/10.1177/1093526618762550