After surgery, the patient recovered successfully. The patient was in good general condition without any clinical symptoms. The echocardiographic examination at 12-month follow-up revealed normal function of the heart, and there was no evidence of tumor recurrence (Fig. 5). The patient signed written informed consent, and the study protocol was approved by the Ethics Committee of West China Hospital of Sichuan University.
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, which makes the current case much rarer. Due to the rarity of the disease, the natural history and pathogenesis of cardiac IMT remain unclear. In general, the clinical symptoms and signs of cardiac IMT are associated with tumor size, location, and rate of growth. One-third of patients with cardiac IMT presented with a constitutional syndrome consisting of fever, weight loss, anemia, thrombocytopenia, hypergammaglobulinemia, and an elevated erythrocyte sedimentation rate.[4,6] Cardiovascular manifestations are the most common way patients present: decreased exercise tolerance, syncope, chest pain, transit ischemic attack, arrhythmia, and sudden death.[4,7,8] After partial or complete resection of the tumor, the clinical outcomes appear to be satisfactory.[9,10] In the current case, although the mass was located in the interventricular septum, the rhythm of the heart was normal suggesting the conduction system was not involved. However, IMT is currently regarded as a neoplasm of intermediate biologic potential, with a 2% risk of metastasis and a 25% of recurrence rate.[11,12] Thus, we completely resected the tumor to prevent further local invasion, which may result in sudden unexpected death.
Diagnosing IMT can be difficult due to the wide morphological and immunohistochemical spectrum and rarity of the disease. Previous literature has demonstrated right atrium and right ventricle predominance. Furthermore, according to gross findings, cardiac IMTs are mainly pedunculated endocardial-based tumors.[4,8,13] In contrast to previous reports, the tumor in the current case is only located in the interventricular septum with a solid gross appearance. The diagnostic approach should start with a thorough history and physical examination. However, symptoms are mainly dependent on the location of the tumor, it is extremely difficult to distinguish between a benign and a malignant lesion. Especially in asymptomatic patients, present day imaging techniques, such as echocardiography, MRI, and computed tomography (CT) are indispensable to make a rapid and precise diagnosis of a cardiac tumor. Echocardiography is regarded as an ideal imaging technique in cardiac disease since it is simple, noninvasive, widely available and has a relatively low cost. Reddy et al  suggested that 3D TTE has an incremental value in the assessment of right ventricular masses allowing for the better identification of the characteristics of the masses compared with CT. Better yet, 3D TTE can provide the measurement of the real dimensions of cardiac tumors and various visual field on different angles and be cropped on any expected plane to evaluate any region of interest contained in the volume acquired.[15,16] In addition, approximately 60 cases of cardiac IMT have been reported, of which 4 were recurrent.[17–20] Among the 4 cases, 2 died of the recurrent IMT.[19,20] Given the recurrence and intermediate biologic potential of cardiac IMT, regular echocardiography was recommended during follow-up. MRI is a complementary technique that provides further information and is used to decide the stage and plan of treatment. Ultimately, the diagnosis of cardiac IMT required a resection specimen postoperatively for histopathologic confirmation, which is the definitive means of diagnosing the cardiac tumor. If necessary, FISH can be performed for further differential diagnosis.
Surgery is considered as the optimal treatment in patients with symptomatic resectable tumors, whereas the role of surgery in patients with asymptomatic cardiac tumors is less clear. This is because cardiac fibromas can remain dormant for many years and even regress. However, due to fatal arrhythmias, surgery is often recommended despite the absence of symptoms. Thus, for cardiac IMTs, complete surgical resection remains the first-line treatment, whenever feasible.
As mentioned earlier, IMT is regarded as a neoplasm of intermediate biologic potential, with 25% of recurrence and a 2% of metastasis. Subtotal tumor is considered as a risk factor for recurrence. According to a review study, cardiac IMT was associated with less frequent recurrence and the ratio reached about 7% in 57 patients. Of the 57 patients, 14 (24.6%) died for several reasons. However, the data may underreport mortality for lack of standardized follow-up. In another review, the author reported that 74% of patients remained asymptomatic after complete surgical resection.
In conclusion, we described a rare cardiac IMT only involving the interventricular septum in a middle-aged adult which has scarcely been reported before. Echocardiography is widely available and is the mainstay for the primary diagnosis of cardiac IMT and regular follow-up examinations. Complete surgical resection of the mass is the first-line treatment even if there is an absence of symptoms.
Conceptualization: Ming-dan Deng, Jun-yang Han, and Hong Tang.
Data curation: Ming-dan Deng and Jun-yang Han.
Formal analysis: Ming-dan Deng and Hong Tang.
Investigation: Ming-dan Deng and Hong Tang.
Methodology: Ming-dan Deng, Jun-yang Han, and Hong Tang.
Project administration: Ke Lin.
Resources: Jun-yang Han, Ke Lin, and Hong Tang.
Software: Ke Lin.
Validation: Ming-dan Deng and Jun-yang Han.
Visualization:Ming-dan Deng, Ke Lin, and Hong Tang.
Writing – original draft: Ming-dan Deng.
Writing – review, and editing: Hong Tang.
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Keywords:Copyright © 2018 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.
cardiac tumor; echocardiography; inflammatory myofibroblastic tumor; three-dimensional echocardiography