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

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.

Differences in investigations of sudden unexpected deaths in young people in a nationwide setting

“…Hence, trying to establish a cause of death is important because family members may be eligible for life-saving therapy. In addition, establishment of a non-inherited cause of death is equally important in the subsequent advice of the family of the deceased [18,19].…”

Imaging of childhood inflammatory myofibroblastic tumor

“…Cardiac involvement of inflammatory myofibroblastic tumor is very rare. Patients with cardiac involvement, ranging in age from 5 weeks to 72 years, have been described in the literature [25]. Cardiac MRI can be used to confirm the presence of a mass, its size, location and extension [5].…”

A Rare Case With Pulmonary and Cardiac Inflammatory Myofibroblastic Tumor

“…1 Although the majority of cardiac tumors follow a benign course after surgical resection, sudden death resulting from cardiac IMT has been reported in 5 cases, including 2 cases as a result of tumor occluding the coronary artery. 2,3 Because cardiac IMT may potentially be fatal if a cardiac valve or the coronary arteries are involved, whenever feasible, complete surgical resection of the tumor remains the mainstay of treatment and e512 Circulation June 2, 2015 seems to have a satisfactory outcome. However, the possibility of recurrence and the long-term prognosis after cardiac surgery have not been determined because of the rarity of these lesions.…”