Malignant Transformation in Von-Meyenburg Complexes: Histologic and Immunohistochemical Clues With Illustrative Cases

Benign developmental defects known as Von-Meyenburg complexes (VMCs) have been postulated to progress to cholangiocarcinoma (CC) in rare instances. Although a gradual, sequential histologic transition from VMCs to CC has been described in some of these cases, the underlying genetic mechanism is yet...

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Veröffentlicht in:Applied immunohistochemistry & molecular morphology 2015-10, Vol.23 (9), p.607-614
Hauptverfasser: Parekh, Vishwas, Peker, Deniz
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container_title Applied immunohistochemistry & molecular morphology
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creator Parekh, Vishwas
Peker, Deniz
description Benign developmental defects known as Von-Meyenburg complexes (VMCs) have been postulated to progress to cholangiocarcinoma (CC) in rare instances. Although a gradual, sequential histologic transition from VMCs to CC has been described in some of these cases, the underlying genetic mechanism is yet to be elucidated. Here, we review the literature to put together a report on the clinicopathologic features and immunohistochemical changes associated with such transformation. We also review the documented association between CC and p16 inactivation, and discuss a possible role for this mechanism to contribute in VMCs to CC progression. In addition, using 2 illustrative cases encountered at our institute, we provide morphologic and immunohistochemical clues that can be used to detect such progression from hamartoma to malignancy. These cases and the accompanying review of literature further solidify the association between VMCs and CC.
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subjects Adult
Aged
Aged, 80 and over
Bile Duct Neoplasms - diagnosis
Bile Duct Neoplasms - genetics
Bile Duct Neoplasms - metabolism
Bile Duct Neoplasms - pathology
Bile Ducts, Intrahepatic - metabolism
Bile Ducts, Intrahepatic - pathology
Cell Transformation, Neoplastic - genetics
Cell Transformation, Neoplastic - metabolism
Cell Transformation, Neoplastic - pathology
Cholangiocarcinoma - diagnosis
Cholangiocarcinoma - genetics
Cholangiocarcinoma - metabolism
Cholangiocarcinoma - pathology
Cyclin-Dependent Kinase Inhibitor p16 - genetics
Cyclin-Dependent Kinase Inhibitor p16 - metabolism
Diagnosis, Differential
Disease Progression
Female
Gene Expression Regulation, Neoplastic
Hamartoma - diagnosis
Hamartoma - genetics
Hamartoma - metabolism
Hamartoma - pathology
Humans
Immunohistochemistry
Male
Middle Aged
title Malignant Transformation in Von-Meyenburg Complexes: Histologic and Immunohistochemical Clues With Illustrative Cases
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