title: Ruptured pericardial perivascular epithelioid cell tumor (PEComa) leading to sudden death: an autopsy case report and review of the literature.
abstract: A 30-year-old man with past medical history of atrial fibrillation/flutter passed away after presenting with sudden-onset cardiac dysfunction. The postmortem examination revealed cardiac tamponade secondary to rupture of a 7.2-cm pericardial perivascular epithelioid cell tumor (PEComa). The tumor grossly appeared to arise from the transverse pericardial sinus and focally penetrated the epicardium of the right atrium. Microscopically, it was composed of predominately spindle cells with low nuclear grade, no pleomorphism, or readily apparent mitoses. Immunohistochemistry revealed cytoplasmic reactivity for HMB-45, desmin, and smooth muscle actin. Electron microscopic findings were characterized by melanosome-like structures intermixed with intermediate filaments and abundant stacked endoplasmic reticulum. The present case is unique among previously reported pericardial/myocardial PEComas as a first example resulting in unexpected cardiac tamponade and sudden cardiac death.
authors.affiliation: Department of Pathology and Immunology, Barnes-Jewish Hospital/St. Louis Children's Hospital, Washington University in St. Louis, St. Louis, MO 63110, United States. Electronic address: lzhang@path.wustl.edu.
authors.forename: Lingxin
authors.initials: L
authors.lastname: Zhang
authors.affiliation: Department of Pathology and Immunology, Barnes-Jewish Hospital/St. Louis Children's Hospital, Washington University in St. Louis, St. Louis, MO 63110, United States. Electronic address: dcarpenter@path.wustl.edu.
authors.forename: Danielle
authors.initials: D
authors.lastname: Carpenter
authors.affiliation: Department of Pathology and Immunology, Barnes-Jewish Hospital/St. Louis Children's Hospital, Washington University in St. Louis, St. Louis, MO 63110, United States. Electronic address: dehner@path.wustl.edu.
authors.forename: Louis P
authors.initials: LP
authors.lastname: Dehner
mesh_terms: D000328:Adult; D001344:Autopsy; D014408:Biomarkers, Tumor / Q000032:analysis; D002305:Cardiac Tamponade / Q000209:etiology / Q000473:pathology; D016757:Death, Sudden, Cardiac / Q000473:pathology*; D017809:Fatal Outcome; D006338:Heart Neoplasms / Q000737:chemistry / Q000150:complications / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D010496:Pericardium / Q000737:chemistry / Q000473:pathology*; D054973:Perivascular Epithelioid Cell Neoplasms / Q000737:chemistry / Q000150:complications / Q000473:pathology*; D012422:Rupture, Spontaneous
chemical_list: D014408:Biomarkers, Tumor
keywords: Autopsy; Perivascular epithelioid cell neoplasms (PEComas); Sudden death