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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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References

  1. Coffin CM, Watterson J, Priest JR, Dehner LP (1995) Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases. Am J Surg Pathol 19(8):859–872

    Article  CAS  PubMed  Google Scholar 

  2. Dahabreh J, Zisis C, Arnogiannaki N, Katis K (1999) Inflammatory pseudotumor: a controversial entity. Eur J Cardiothorac Surg 16(6):670–673

    Article  CAS  PubMed  Google Scholar 

  3. Karnak I, Senocak ME, Ciftci AO, Cağlar M, Bingöl-Koloğlu M, Tanyel FC, Büyükpamukçu N (2001) Inflammatory myofibroblastic tumor in children: diagnosis and treatment. J Pediatr Surg 36(6):908–912

    Article  CAS  PubMed  Google Scholar 

  4. Coffin CM, Humphrey PA, Dehner LP (1998) Extrapulmonary inflammatory myofibroblastic tumor: a clinical and pathological survey. Semin Diagn Pathol 15(2):85–101

    CAS  PubMed  Google Scholar 

  5. Kapusta LR, Weiss MA, Ramsay J, Lopez-Beltran A, Srigley JR (2003) Inflammatory myofibroblastic tumors of the kidney: a clinicopathologic and immunohistochemical study of 12 cases. Am J Surg Pathol 27(5):658–666

    Article  PubMed  Google Scholar 

  6. Anvari MS, Soleimani A, Abbasi A, Boroumand MA, Marzban M, Karimi AA, Yazdanifard P, Shirani S, Sahebjam M (2009) Inflammatory myofibroblastic tumor of the right ventricle causing tricuspid valve regurgitation. Tex Heart Inst J 36(2):164–167

    PubMed  Google Scholar 

  7. Burke A, Li L, Elaine Kling E, Kutys R, Virmani R, Miettnen M (2007) Cardiac inflammatory myofibroblastic tumor: A “benign” neoplasm that may result in syncope, myocardial infarction, and sudden death. Am J Surg Pathol 31(7):1115–1122

    Article  PubMed  Google Scholar 

  8. Gonzalez-Crussi F, Vanderbilt BL, Miller JK (1975) Unusual intracardiac tumor in a child. Inflammatory pseudotumor or “granulomatous’ variant of myxoma? Cancer 36(6):2214–2226

    Article  CAS  PubMed  Google Scholar 

  9. Lam KY, Dickens P, Chan AC (1993) Tumors of the heart. A 20-year experience with a review of 12, 485 consecutive autopsies. Arch Pathol Lab Med 117(10):1027–1031

    CAS  PubMed  Google Scholar 

  10. Uzun O, Wilson DG, Vuianic GM, Parsons JM, Giovanni JVD (2007) Cardiac tumor in children. Orphanet J Rare Dis 2:11–14

    Article  PubMed  Google Scholar 

  11. Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T (2005) Cardiac tumours: diagnosis and management. Lancet Oncol 6(4):219–228

    Article  PubMed  Google Scholar 

  12. Mariano A, Pita A, Leon R, Rossi R, Gouveia R, Teixeira R, Anjos R, Menezes I, Martins FM (2009) Primary cardiac tumors in children: a 16-year experience. Rev Port Cardiol 28(3):279–288

    PubMed  Google Scholar 

  13. Vinter S, Isaksen C, Vesterby A (2005) Sudden cardiac death in a young woman: tumor of the atrioventricular (AV) node or citalopram intoxication? Am J Forensic Med Pathol 26(4):349–351

    Article  PubMed  Google Scholar 

  14. Patel J, Sheppard MN (2009) Cystic tumor of the atrioventricular node: three cases of sudden death. Int J Leg Med (in press)

  15. Fracasso T, varchmin-schltheiss K (2009) Sudden death due to pulmonary embolism from right atrial myxoma. Int J Leg Med 123:157–159

    Article  CAS  Google Scholar 

  16. Dickens P, Lam AK (1991) Sudden death associated with cardiac inflammatory pseudotumor. Forensic Sci Int 49:89–93

    Article  CAS  PubMed  Google Scholar 

  17. Rose AG, McCormick S, Copper K, Titus JL (1996) Inflammatory pseudotumor (plasma cell granuloma) of the heart. Report of two cases and literature review. Arch Pathol Lab Med 120(6):549–554

    CAS  PubMed  Google Scholar 

  18. Krishna L, Ng WL, Chachlani N (2001) Inflammatory pseudotumor of the heart causing aortic regurgitation. Ann Thorac Surg 71(4):1361–1363

    Article  CAS  PubMed  Google Scholar 

  19. Gleason BC, Hornick JL (2008) Inflammatory myofibroblastic tumours: where are we now? J Clin Pathol 61:428–437

    Article  CAS  PubMed  Google Scholar 

  20. Nascimento AF, Ruiz R, Hornick JL, Fletcher CDM (2002) Calcifying fibrous ‘pseudotumor’. Int J Surg Pathol 10(3):189–196

    Article  PubMed  Google Scholar 

  21. Cook JR, Dehner LP, Collin MH et al (2001) Anaplastic lymphoma kinase (ALK) expression in the inflammatory myofibroblatic tumor: a comparative immmunohistochemistry study. Am J Surg Pathol 25:1364–1371

    Article  CAS  PubMed  Google Scholar 

  22. Tazelaar H, Manabe T (2004) Inflammatory myofibroblastic tumor. In: Travis WDBE, Muller-Hermelink HK, Harris CC (eds) Tumors of the Lung, Pleura, Thymus, and Heart. ARC Press, Lyon, pp 273–281

    Google Scholar 

  23. Hartyanszky IL, Kadar K, Hubay M (2000) Rapid recurrence of an inflammatory myofibroblastic tumor in the right ventricular outflow tract. Cardiol Young 10:271–274

    Article  CAS  PubMed  Google Scholar 

  24. Grebenc ML, Rosado De Christenson ML, Burke AP, Green CE, Galvin JR (2000) Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics 20:1073–1103

    CAS  PubMed  Google Scholar 

  25. Smith DN, Shaffer K, Patz EF (1998) Imaging features of nonmyxomatous primary neoplasms of the heart and pericardium. Clin Imaging 22:15–22

    Article  CAS  PubMed  Google Scholar 

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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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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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