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

Autor: Wilken, Silvana, Thevathasan, Tharusan, Kamali, Can, Guillot, Adrien, Ihlow, Jana, Fehrenbach, Uli, Danyel, Magdalena, Pratschke, Johann, Tacke, Frank, Krenzien, Felix
Zdroj: Clinical Journal of Gastroenterology; Apr2024, Vol. 17 Issue 2, p300-306, 7p
Abstrakt: 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. [ABSTRACT FROM AUTHOR]
Databáze: Complementary Index