An Unexpected Cause of Respiratory Distress and Cyanosis: Cardiac Inflammatory Myofibroblastic Tumor

Abstract

Inflammatory myofibroblastic tumor is an uncommon spindle cell tumor, occurring mainly in children and young adults. It is an extremely rare cardiac tumor especially patients under 1 year. Although it is benign, the tumor may be very aggressive locally. The diagnosis of this unusual pediatric cardiac tumor without pathologic specimens is difficult. We report a rare case of inflammatory myofibroblastic tumors of the right ventricle in a 7-month-old girl presenting with respiratory distress and cyanosis.

Febrile Illness in an Infant With an Intracardiac Inflammatory Myofibroblastic Tumor

“…These are differentiated from IMTs based on histology and extent of invasion into the myocardium. 9,10 IMTs appear histologically similar to myosarcomas but can be differentiated on the basis of their having less cellular pleomorphism, atypia, and fewer mitotic figures 1,10 and not invading into the myocardium. ALK-1 is positive in 35% of IMTs, 11 although a negative result does not rule out the diagnosis because several intracardiac IMTs have previously stained negative.…”

“…Intracardiac IMTs are generally described as benign tumors; however, depending on their location, the initial presentation may involve heart failure or sudden death. 1 In addition to cardiac signs and symptoms, the clinical presentation of IMTs may also include constitutional signs such as fever, anemia, and elevated inflammatory markers. This case report reviews the diagnosis and management of IMTs, as well as the histopathologic features of this rare tumor type.…”

“…Including our case, 49 cases of intracardiac IMTs have been reported across all age groups (see Supplemental Information). 1 Of these, 33 have been reported in the pediatric population, with approximately half of this group involving children <12 months of age. 2 Although IMTs are generally described as benign tumors, a small number demonstrate neoplastic properties, such as local recurrence after resection.…”

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.…”