title: Demonstration of MyoD1 expression in oncocytic cardiomyopathy: report of two cases and review of the literature.
abstract: Oncocytic cardiomyopathy is a rare arrhythmogenic disorder usually associated with female sex, difficult-to-control arrhythmias, or sudden death of infants and children. Morphologically, it is characterized by the presence of oncocytic cells, which are diffusely distributed or form the nodular structures within the myocardium, occasionally involving the valves, with a large number of mitochondria in cytoplasms. We present two cases of oncocytic cardiomyopathy. The first case had a fatal clinical outcome, and the other case was surgically treated. The nuclear expression of skeletal muscle transcription factor MyoD1 was demonstrated in the first case, supporting the theory that oncocytic cardiomyopathy is a conduction system developmental disorder. To confirm this hypothesis, it is necessary to further investigate myogenic transcription factor program in human cardiac conduction system cells.
authors.affiliation: Department of Pathology, Faculty of Medicine, Masaryk University and University Hospital, Brno, Czech Republic.
authors.forename: S
authors.initials: S
authors.lastname: Hotárková
authors.forename: M
authors.initials: M
authors.lastname: Hermanová
authors.forename: V
authors.initials: V
authors.lastname: Povýsilová
authors.forename: K
authors.initials: K
authors.lastname: Dvorák
authors.forename: J
authors.initials: J
authors.lastname: Feit
authors.forename: Z
authors.initials: Z
authors.lastname: Lukás
authors.forename: L
authors.initials: L
authors.lastname: Kren
authors.forename: P
authors.initials: P
authors.lastname: Vit
authors.forename: H
authors.initials: H
authors.lastname: Jicínská
authors.forename: B
authors.initials: B
authors.lastname: Hucín
mesh_terms: D009202:Cardiomyopathies / Q000378:metabolism* / Q000473:pathology*; D002648:Child; D005260:Female; D006330:Heart Defects, Congenital / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D008854:Microscopy, Electron; D017570:MyoD Protein / Q000096:biosynthesis*; D009206:Myocardium / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D024862:Oxyphil Cells / Q000473:pathology*
chemical_list: D017570:MyoD Protein; C057363:MyoD1 myogenic differentiation protein