Inflammatory Myofibroblastic Tumor of the Right Atrium

Abstract

Cardiac inflammatory myofibroblastic tumor (IMT) is a rare entity and is associated with distinct clinical, pathological and molecular features. The clinical behavior, natural history, biological potential, management and prognosis of such tumors are unclear. We present herewith an adolescent girl who presented with similar entity involving the junction of the right atrium and the inferior vena cava (IVC) in association with thrombocytosis and IVC thrombosis leading to obstruction of blood flow. Diagnostic tools included imaging and immuno-histopathology studies. Surgical management included resection of the tumor and thrombo-embolectomy of the IVC under cardiopulmonary bypass. This case is unique due to association of complete obstruction of IVC caused by the strategic location of the tumor, thrombosis of vena cava and association of thrombocytosis. These features have not been reported yet in relation to the cardiac IMT. This report will help in better understanding and management of similar cases in terms of planning cannulation of femoral veins or application of total hypothermic circulatory arrest during cardiopulmonary bypass and prompt us to look for recurrence or metastasis during follow up using echocardiography and laboratory investigations. The possibility of IMT should be kept in the differential diagnosis of cardiac tumors especially in children and adolescents.

The Right Inferior Pulmonary Vein Related Inflammatory Myofibroblastic Tumor in an Adult Case

“…IMT of cardiac origin is mostly in the form of polypoid lesion with small myocardial infiltrations [4]. There is no specific symptom and it can show signs according to the location site [5]. IMT associated withheart valves and coronary arteries can progress fatally [4].…”

“…There is no specific distinguishing characteristic in cardiac lesions. Echocardiography is generally used in diagnosis [4,5]. While the ones located in the left atrium may lead to pulmonary edema, the ones located in the right atrium may cause vena cava inferior thrombosis, liver congestion or vena cava superior disease [4,5].…”

“…Videothoracoscopic surgical resection with stable surgical margins is a good option in solitary pulmonary nodules [7]. Surgical excision is a diagnostic and treatment option in the ones with cardiac origin [5].…”

Cardiac Inflammatory Myofibroblastic Tumor

“…The various synonyms were taken into account, identifying 57 cases of IMT. Table 1 summarizes all 57 cases, 6 –52 including the case at our institution. This represents the most comprehensive review of cardiac IMTs published to date.…”

Inflammatory Myofibroblastic Tumor of the Aortic Valves Causing Sudden Cardiac Death: A Case Report and Review of the Literature

“…It may occur throughout the body and at any age, but the majority of the cases have been reported in the abdominal or pelvic cavity of children and young adults [1–3]. Inflammatory myofibroblastic tumor of the heart is rare, with fewer than 60 cases being reported in the literature under various designations, including IMT, plasma cell granuloma, inflammatory pseudotumor, and inflammatory fibrosarcoma [1,4–47]. It typically presents as an endocardium-based polypoid mass and may arise in the atrium, ventricle, and/or cardiac valve.…”