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Sudden unexpected death due to inflammatory myofibroblastic tumor of the heart: a case report and review of the literature

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Abstract

Inflammatory myofibroblastic tumor (IMT) or inflammatory pseudotumor is a rare primary cardiac tumor that may result in sudden death. We report a sudden unexpected death due to occlusion of the coronary arteries by IMT arising from the left coronary cusp of the aortic valve. An 8-year-old child suddenly woke up from his sleep with complaint of severe chest pain to his parents, and shortly he became unresponsive. He expired 40 min later in the hospital despite resuscitation efforts. The postmortem examination revealed a 2.5 × 2 × 1-cm mass composed of multiple entangled slender cylindrical fronts, filling the coronary sinus and obstructing the coronary ostia. The patient had complained of recurrent chest pains about 2 weeks prior to his death. Echocardiogram was conducted on the patient but did not recognize the mass. Histological examination of the mass established the diagnosis of primary cardiac IMT. The detailed pathological findings are described. In addition, the literature is reviewed, and pathogenesis, clinical presentation, and the importance of forensic autopsy examination are discussed.

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Authors and Affiliations

  1. Department of Pathology, University of Maryland School of Medicine, Baltimore, USA

    Ling Li, Allen Burke & David R. Fowler

  2. Department of Pediatrics, University of Maryland School of Medicine, 655 W. Baltimore Street, BRB 13-013, Baltimore, MD, 21201, USA

    Ling Li, H. Ronald Zielke & David R. Fowler

  3. Department of Surgery, Cancer Institute and Hospital, Chinese Academy of Medical Science, Beijing, China

    Ling Li & Jie He

  4. Key Laboratory of Evidence Science, Division of Forensic Pathology, China University of Political Science and Law, Beijing 100040, China

    Ling Li, Lin Chang & David R. Fowler

Authors
  1. Ling Li
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  2. Allen Burke
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  3. Jie He
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  4. Lin Chang
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  5. H. Ronald Zielke
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  6. David R. Fowler
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Corresponding author

Correspondence to Ling Li.

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Fig. 5

Movat pentachrome stain of area is shown in the figure demonstrating proteoglycan-rich matrix (green). (Original magnification ×20) (GIF 680 kb)

High resolution image file (TIFF 4.63 mb)

Fig. 6

The tumor cells were negative for CD 34 but small vessel endothelial cells were positive (CD 34 immunohistochemical stain; original magnification ×40) (GIF 787 kb)

High resolution image file (TIFF 4.71 mb)

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Li, L., Burke, A., He, J. et al. Sudden unexpected death due to inflammatory myofibroblastic tumor of the heart: a case report and review of the literature. Int J Legal Med 125, 81–85 (2011). https://doi.org/10.1007/s00414-010-0496-7

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  • DOI: https://doi.org/10.1007/s00414-010-0496-7

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