Ruptured pericardial perivascular epithelioid cell tumor (PEComa) leading to sudden death: An autopsy case report and review of the literature

Lingxin Zhang, Danielle Carpenter, Louis P. Dehner

Research output: Contribution to journalArticle

2 Scopus citations

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.

Original languageEnglish
Pages (from-to)63-66
Number of pages4
JournalCardiovascular Pathology
Volume25
Issue number1
DOIs
StatePublished - Jan 1 2016

Keywords

  • AML angiomyolipoma
  • Abbreviations PEComa perivascular epithelioid cell tumor
  • LAM lymphangiomyomatosis
  • TSC tuberous sclerosis complex

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