Patient with a novel syndrome with multiple benign hepatic lesions and extrahepatic neoplasms

Simultaneous occurrence of benign hepatic lesions of different types is a sporadic phenomenon. To the best of our knowledge, we report the first clinical case of a syndrome with simultaneous manifestations of three different entities of benign liver tumors (hepatocellular adenoma, focal nodular hype...

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Bibliographic Details
Published in:Clinical journal of gastroenterology 2024-04, Vol.17 (2), p.300-306
Main Authors: Wilken, Silvana, Thevathasan, Tharusan, Kamali, Can, Guillot, Adrien, Ihlow, Jana, Fehrenbach, Uli, Danyel, Magdalena, Pratschke, Johann, Tacke, Frank, Krenzien, Felix
Format: Article
Language:English
Subjects:
Abdominal Surgery
Adenoma - pathology
Case Report
Colorectal Surgery
Focal Nodular Hyperplasia - complications
Focal Nodular Hyperplasia - diagnosis
Focal Nodular Hyperplasia - pathology
Gastroenterology
Hemangioma - complications
Hemangioma - pathology
Hepatology
Humans
Liver - pathology
Liver Neoplasms - pathology
Medicine
Medicine & Public Health
Surgical Oncology
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Summary:Simultaneous occurrence of benign hepatic lesions of different types is a sporadic phenomenon. To the best of our knowledge, we report the first clinical case of a syndrome with simultaneous manifestations of three different entities of benign liver tumors (hepatocellular adenoma, focal nodular hyperplasia and hemangioma) with a novel mutation detected in the liver adenoma and in the presence of a number of further extrahepatic organ neoplasms. Furthermore, we describe for the first time the presence of liver epithelial cells of hepatocytic phenotype expressing cytokeratin 7 (CK7) at the border of the adenoma. These findings may be important for explaining pathogenesis of benign as well as malignant tumors based on genetic and histopathological features.
ISSN:1865-7257
1865-7265
1865-7265
DOI:10.1007/s12328-023-01899-3