Abstract:Cardiac inflammatory myofibroblastic tumor (IMT) is a rare entity affecting predominantly infants, children, and young adults. Although most tumors have a benign clinical course after complete surgical resection, some have significant clinical effects. We report the case of a 9-year-old girl who had sudden cardiac death as a result of occlusion of the left circumflex coronary artery. A review of 57 cases of cardiac IMTs reported in the literature in terms of epidemiology, clinical presentation, histologic and … Show more
“…We were unable to investigate any complaints before collapse in the present case. Previous reports showed six cases of sudden death associated with cardiac IMT ( 14 - 19 ). Among them, five cases were caused by myocardial ischemia because of occlusion of the coronary arteries by the IMT.…”
The patient was a 34-year-old woman who suddenly collapsed. On arrival, she was in cardiac arrest. Cardiac ultrasound revealed cardiac tamponade; thus, urgent thoracotomy with pericardiotomy was performed. Spontaneous circulation was temporarily obtained; however, her circulation was not stabilized, and she ultimately died. An autopsy revealed a pericardial inflammatory myofibroblastic tumor (IMT) causing bloody cardiac tamponade. There were no signs of cardiac rupture, myocardial infarction or aortic dissection. We reported the first case of fatal bloody cardiac tamponade due to pericardial IMT in an adult. An autopsy is important for clarifying the etiology in cases of fatal cardiac tamponade of unknown cause.
“…We were unable to investigate any complaints before collapse in the present case. Previous reports showed six cases of sudden death associated with cardiac IMT ( 14 - 19 ). Among them, five cases were caused by myocardial ischemia because of occlusion of the coronary arteries by the IMT.…”
The patient was a 34-year-old woman who suddenly collapsed. On arrival, she was in cardiac arrest. Cardiac ultrasound revealed cardiac tamponade; thus, urgent thoracotomy with pericardiotomy was performed. Spontaneous circulation was temporarily obtained; however, her circulation was not stabilized, and she ultimately died. An autopsy revealed a pericardial inflammatory myofibroblastic tumor (IMT) causing bloody cardiac tamponade. There were no signs of cardiac rupture, myocardial infarction or aortic dissection. We reported the first case of fatal bloody cardiac tamponade due to pericardial IMT in an adult. An autopsy is important for clarifying the etiology in cases of fatal cardiac tamponade of unknown cause.
“…Cardiac IMTs despite being a rare occurrence poses a serious risk of significant morbidity and sudden cardiac death, 4 with reports of extension into great vessels including the pulmonary artery. 5 The etiology of IMTs has been debated, and most common explanations include immunologic proliferation in genetically susceptible populations, altered response to infections such as HHV6, and fibroblastic proliferation.…”
Inflammatory myofibroblastic tumors (IMTs) represent mesenchymal tumors that occur in the lungs, abdomen, or pelvis. Cardiac IMTs are rare, usually right-sided, and when left-sided can cause sudden cardiac death by coronary occlusion. We report a child with symptoms of embolization to the right kidney and the femoral artery, and a mobile mass in the left atrium attached to the mitral valve. Upon surgical removal, histopathology revealed IMT. Our case illustrates a unique presentation of systemic thromboembolism.
“…IMTs are rarely involved in heart accounting for less than 5% of primary cardiac tumors. Cardiac IMTs have a predilection for patients less than 20 years old; less than 10 cases reported occurred in older adults, [5] which makes the current case much rarer. Due to the rarity of the disease, the natural history and pathogenesis of cardiac IMT remain unclear.…”
Section: Discussionmentioning
confidence: 99%
“…Thus, for cardiac IMTs, complete surgical resection remains the first-line treatment, whenever feasible. [5]…”
Section: Discussionmentioning
confidence: 99%
“…In another review, the author reported that 74% of patients remained asymptomatic after complete surgical resection. [5]…”
Rationale:Cardiac inflammatory myofibroblastic tumor (IMT) is a rare primary cardiac tumor which is currently considered as a low-grade neoplasm. The tumor has a predilection in infants and adolescents and primarily occurs as an endocardial-based cavitary mass. However, cardiac IMT that only involves the interventricular septum in middle-aged adults is extremely rarely reported. Considering its infrequency, we report a rare clinical case, with the aim of sharing our experience during the diagnostic procedures.Patient concerns:A 45-year-old, previously healthy female, with no medical history was admitted to the outpatient clinic due to the identification of an abnormal radiographic finding during a routine health examination.Diagnosis:Transthoracic echocardiography (TTE) revealed a 3.5 cm × 4.0 cm × 4.5 cm heterogeneous mass in the interventricular septum. Color Doppler echocardiography detected sparse blood flow signals inside the mass. Magnetic resonance imaging (MRI) confirmed a hyperintense T2-weighted, isointense T1-weighted mass. Three-dimensional (3D) TTE demonstrated a spherical mass in the middle part of the interventricular septum. Postoperative histopathological examinations revealed a mesenchymal tumor composed of scattered spindle myofibroblasts with a myxoid atypia, associated with infiltration of lymphocytes and plasma cells.Interventions:Complete tumor resection was successfully performed via median sternotomy under general anesthesia.Outcomes:After surgery, the patient recovered successfully. The patient was in good general health without any clinical symptoms. The echocardiographic examination at the 12-month follow-up period revealed normal function of the heart, and there was no evidence of tumor recurrence.Lessons:To our knowledge, cardiac IMT only the involving interventricular septum in a middle-aged adult has never been previously reported before. Echocardiography plays a critical role in establishing the primary diagnosis of cardiac IMT and evaluating regular follow-up examinations. Complete surgical resection of the mass is considered the first-line treatment despite the absence of symptoms.
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